Seminar 5 - Smoking Associated Lung Disease

Question 1

What is the difference between type 1 and 2 respiratory failure

Answer 1

Type 1 has just hypoxia with low or normal CO2

Type 2 has hypoxia with hypercapnia

Question 2

Describe the course of subacute fibrotic lung diseases

Answer 2

Have a resolving, remitting, relapsing or progressive course

Question 3

In which lung disease would you see increased serum ACE and Ca2

Answer 3

Sarcoidosis

Question 4

How does CTEPH present on V/Q scan

Answer 4

Will have one or more segmental or larger unmatched perfusion defects

Question 5

Describe the pathogenesis of Group 3 PH

Answer 5

Lung disease leads to hypoxia
Pulmonary vasoconstriction occurs in response to this.
This process is normally reversible but sustained hypoxia activates further mediators which leads to remodelling and increase in pulmonary vascular resistance.
As a result, the pressure in the vessels rises and you get PH

Question 6

What is anthracnosis

Answer 6

Form of pneumoconiosis
Most innocuous coal-induced lesion
Seen to some extent in city living & smokers)
Usually doesn’t cause fibrosis but can if severe

Question 7

What causes the diaphragm paralysis seen in lung cancer

Answer 7

Phrenic nerve invasion

Question 8

What proportion of COPD patients have PH

Answer 8

10-30%

Question 9

List the symptoms of Horner’s syndrome

Answer 9

Unilateral ptosis, miosis, anhidrosis and enophthalmos

Question 10

How do you differentiate between obstructive and restrictive lung disease

Answer 10

History and examination can help but often considerable symptom overlap
Main way is via pulmonary function tests such as spirometry

Question 11

What are asbestos bodies

Answer 11

Long, thin asbestos fibres with translucent centre and gold-brown colour
Consists of asbestos fibres coated with iron-containing proteinaceous material

Question 12

How do you name benign tumours of mesenchymal cells

Answer 12

Suffix “-oma” is attached to the name of the cell type from which the tumor arises
e.g. lipoma

Question 13

What determines the outcome of Group 1 PAH

Answer 13

The underlying cause, severity and available treatment options
Also a list of prognostic factors (other card)

Question 14

Describe the prevalence of intrinsic lung diseases

Answer 14

Overall prevalence of 3-6 cases/100,000 people (US)

Idiopathic pulmonary fibrosis (IPF) prevalence in UK is 50/100,000

Question 15

What is the most common cause of cor pulmonale

Answer 15

COPD

Question 16

List examples of chest wall disorders

Answer 16

Severe obesity

Pleural diseases (e.g. trapped lung, scarring, large pleural effusions, chronic empyema)

Kyphoscoliosis

Neuromuscular diseases (e.g. MG, ALS, myopathy)

Question 17

What can decrease mortality in COPD

Answer 17

Smoking cessation has been shown to decrease mortality

Question 18

Which pneumoconiosis is not associated with an increased risk of TB or cancer development?

Answer 18

Coal workers’ pneumoconiosis
(when considering disease on its own – non-smokers)

Indoor smoky coal can increase risk but rare in western world

Question 19

Which HLA genotypes are associated with sarcoidosis

Answer 19

HLA-A1 and HLA-B8

Question 20

In which lung disease would you see

ground-glass opacities on CXR

Answer 20

Pulmonary fibrosis

Question 21

Describe the prevalence of sarcoidosis

Answer 21

in US: 10-40/100,000people.
Prevalenceof this disease is hard to determine as hard to diagnose
10x more common in African Americans than Caucasians

Question 22

How does Respiratory bronchiolitis-associated interstitial lung disease present

Answer 22

Patients will have significant pulmonary symptoms, abnormal lung function and imaging abnormalities

Term reserved for these patients

Question 23

What are the 2 main components of every tumour

Answer 23

Neoplastic cells that constitute the tumor parenchyma

Reactive stroma made up of connective tissue, blood vessels and cells of the adaptive and innate immune system

Question 24

Describe V/Q mismatch

Answer 24

It is the most common cause of hypoxemia
The levels ventilation and blood flow do not match - not sufficient gas exchange

Caused by reduced ventilation from airway, interstitial lung disease or reduced perfusion in PE (blood cannot reach ventilated alveoli)

Question 25

What environmental factors increase you risk of COPD

Answer 25

``` Long term pollution exposure Cigarettes - biggest cause Airway hyper-reactivity IV drug use - pulmonary vascular damage Immunodeficiency e.g. HIV Vasculitis - HVUS Connective tissue disorders e.g. Marfans, Ehler's Danlos ```

Question 26

What is the typical mechanism of death in COPD

Answer 26

Fatal due to heart failure or of respiratory failure due to superimposed infection

Question 27

What signs may be found on examination of a patient with CTEPH

Answer 27

may hear reduced or fixed splitting of the second heart sound, louder pulmonary valve closure or feel a right ventricular heave.

Question 28

Which diseases affecting the lung parenchyma can cause cor pulmonale

Answer 28

``` COPD Bronchiectasis Pulmonary Fibrosis Severe, chronic asthma Lung resection ```

Question 29

Describe the pathogenesis of atopic asthma

Answer 29

Following exposure to allergen, Th2 cells produce cytokines (interleukins 5, 4 and 13) which activate leukocytes such as eosinophils and stimulate production of IgE The T cells and epithelial cells recruit more eosinophils, perpetuating this reaction The IgE produced by B cells binds to FC receptors on mast cells Repeated exposure to the allergen causes mast cells to degranulate The early phase hypersentivity reaction kicks in followed by the late phase

Question 30

How can infections contribute to asthma

Answer 30

Children with LRTI have a 10-30 fold increased risk of developing persistent or severe asthma Infections also exacerbate asthma

Question 31

How does chromatin appear in the nuclei of malignant cells

Answer 31

Coarsely clumped and distributed along the nuclear membrane | More darkly stained than normal (hyperchromatic)

Question 32

Which of the components of COPD causes reversible damage

Answer 32

Bronchoconstriction due to inflammation accounts for some reversibility Airflow obstruction in emphysema is often irreversible

Question 33

Which lung cancers could be classed as T4

Answer 33

Tumour > 7cm OR Involvement of the mediastinum, heart, great vessels, trachea, oesophagus, recurrent laryngeal nerve, vertebral body or carina OR Separate tumour nodules in a different ipsilateral lobe

Question 34

How does silicosis present on CXR

Answer 34

Fine nodularity in upper zones Eggshell calcification (Ca surrounding zone lacking calcification) Seen as thin sheets of calcification in lymph nodes.

Question 35

In which lung disease would you see polyploid plugs of loose connective tissue called Masson bodies

Answer 35

Cryptogenic Organising Pneumonia | Also called Bronchiolitis obliterans organising pneumonia (BOOP)

Question 36

What causes Group 2 PAH

Answer 36

Left sided heart disease

Question 37

How do you diagnose pulmonary haemorrhage

Answer 37

Bloods - FBC, coagulation studies, serology Bronchoscopy - assess bleeding site Lung biopsy - carried out if the less invasive tests cannot find a cause Further tests may be carried out if a specific cause is suspected

Question 38

Which lung cancers could be classed as N3

Answer 38

Metastasis to contralateral mediastinal or hilar lymph nodes, ipsilateral or contralateral scalene, or supraclavicular lymph nodes

Question 39

Why is asthma a disease of industrialized/urban environments (describe the 2 main theories)

Answer 39

Cities contain many allergens that can initiate a Th2 response such as airborne pollutants Hygiene hypothesis - City life tends to limit children’s exposure to antigens which could provide a protective effect against atopy

Question 40

List the subtypes of non-small cell lung cancer

Answer 40

Adenocarcinoma - most common Squamous cell Large cell undifferentiated Others such as carcinoid

Question 41

Describe the progression of Group 1 PAH

Answer 41

Progressive and sustained rise in pulmonary vascular pressure. Typically caused by vasoconstriction, remodelling and thrombosis in the small pulmonary arteries and arterioles. Can progress to right heart failure

Question 42

For a coagulation disorder to lead to pulmonary haemorrhage, what else is typically needed

Answer 42

Often need a precipitant like infection

Question 43

What are the clinical features of pulmonary haemorrhage

Answer 43

Classic symptoms: dyspnoea, haemoptysis and iron deficiency anaemia Exact clinical presentation varies depending on the underlying cause (will have symptoms of that disease Chronic bleeding may cause fever, cough, weight loss and tiredness

Question 44

List the main signs of asthma

Answer 44

Diurnal variability Triggers – exercise, allergens, infection, cold weather, drugs (NSAIDS) Associated atopy (eczema, hayfever etc) - due to increased IgE levels Blood eosinophilia > 3% Responsive to steroids or beta-agonists FMHx of asthma

Question 45

Which people get respiratory bronchiolitis-associated interstitial lung disease

Answer 45

Typically current smokers with >30 pack year history in 40/50s

Question 46

What types of radiation can increase risk of lung cancer

Answer 46

Uranium – 4x risk in non-smoking Uranium miners Radon gas – Increased risk of exposure in underground workers and places with high soil concentration Ionising radiation – Increased incidence in atomic bomb survivors, as well as those involved in the Chernobyl clean-up

Question 47

What is chronic bronchitis

Answer 47

Defined clinically as the presence of a chronic productive cough for 3 months during each of 2 consecutive years with other causes of cough being excluded

Question 48

Describe the epidemiology of Group 4 PH (basically CTEPH)

Answer 48

3-5 cases per 100,000 in the US and Europe. Exact incidence after PE is uncertain though 75% of CTEPH patients had a previous PE. Often underdiagnosed No sex difference

Question 49

What may you see on chest examination of a patient with COPD

Answer 49

Hyperinflation (barrel chest) Wheezing – Frequently heard on forced and unforced expiration Diffusely decreased breath sounds Hyperresonance on percussion Prolonged expiration Coarse crackles beginning with inspiration in some cases

Question 50

Discuss the mortality rates in COPD

Answer 50

Globally, it is estimated that 3.17 million deaths were caused by the disease in 2015 (that is, 5% of all deaths globally in that year

Question 51

List the macroscopic features of complex coal worker's pneumoconiosis

Answer 51

Causes progressive massive fibrosis | Intensely blackened scars 1cm+; usually multiple

Question 52

Is is chronic bronchitis or emphysema that affects the elastic recoil of the lungs

Answer 52

Emphysema - reduces it | Normal in CB

Question 53

Describe the prognosis of respiratory failure

Answer 53

Respiratory failure itself doesn’t have an exact prognosis because it is determined by the underlying cause

Question 54

What is asbestos

Answer 54

A family of crystalline hydrated silicates Different forms such as serpentine chrysotile form (90% of asbestos in industry) and amphiboles form (less prevalent, more pathogenic) Associated lung disease

Question 55

How does asbestosis present on CXR

Answer 55

Circumscribed densities

Question 56

List common asthma co-morbidities

Answer 56

``` Rhinitis and rhinosinusitis  Obesity Obstructive sleep apnoea GORD May overlap with COPD Mental health disorders (anxiety/depression) ```

Question 57

How do you measure pulmonary arterial pressure

Answer 57

Cardiac catherisation

Question 58

Describe the characteristics of usual interstitial pneumonia

Answer 58

Fibrosis seen on histology Non-uniform (patchy), variegated lung injury Alternating areas of healthy lung, interstitial inflammation, fibrosis and honeycomb change

Question 59

Discuss the common mechanism of death in COPD

Answer 59

Airflow obstruction is associated with increased mortality, even with mild impairment In mild to moderate COPD, majority of deaths are due to cardiovascular disease or lung cancer, As COPD severity increases, respiratory deaths are increasingly common

Question 60

How do you name a cancer if the cells are of unknown origin

Answer 60

Designated merely as undifferentiated malignant tumors | Only occurs in around 2% of cases

Question 61

What are some of the possible carcinogens in tobacco

Answer 61

Polycyclic aromatic hydrocarbons Phenol derivatives Nitrosamines Radioactive elements

Question 62

Describe the early phase reaction of atopic asthma

Answer 62

Consists of: Bronchoconstriction - triggered by direct stimulation of parasympathetic receptors through reflexes triggered by mediators produced by mast cells Increased mucus production Variable degrees of vasodilatation and increased vascular permeability

Question 63

What are the characteristics of Desquamative interstitial pneumonia (DIP) and Respiratory bronchiolitis-associated interstitial lung disease

Answer 63

Large collections of macrophages in airspaces of a current or previous smoker Called smokers macrophages Some macrophages have lamellar bodies (contain surfactant) within phagocytic vacuoles. Alveolar septa thickened by sparse inflammatory infiltrate of lymphocytes, plasma cells and some eosinophils

Question 64

List the macroscopic features of Group 1 PAH

Answer 64

Hypertrophy of intima, media and adventitia of pulmonary arteries

Question 65

What are the two aetiological categories of restrictive lung disease

Answer 65

Chest wall disorders | Chronic infiltrative and interstitial disease

Question 66

Where are you most likely to find pleural plaques in asbestosis

Answer 66

Found on anterior and posterior-lateral aspects of parietal pleura and over diaphragm domes

Question 67

Group 1 PAH is a diagnosis of exclusion - true or false

Answer 67

True | You must differentiate it from the other WHO groups

Question 68

List some of the common medication side effects seen in the treatment of asthma

Answer 68

``` Rapid heartbeat Hoarseness Throat irritation (ICS) Oral yeast infections (ICS) Insomnia (theophylline) Gastroesophageal reflux (theophylline) ```

Question 69

Describe the typical course of silicosis

Answer 69

Follows a slow & insidious course (10-30y after exposure) However it can be accelerated (<10y) or rapid (weeks-months after intense exposure to fine dust high in silica).

Question 70

Describe the structure of the pleural plaques seen in asbestosis

Answer 70

Well-circumscribed plaques of dense collagen Often calcified Don’t contain asbestos bodies but rarely occur w/o exposure to asbestos.

Question 71

Describe the epidemiology of Group 2 PAH

Answer 71

Around 6.5 million people in Europe have heart failure | Of those with a reduced ejection fraction, 60% have this PH on presentation

Question 72

Neoplasms are typically soft and fleshy

Answer 72

True | Connective tissue is usually scant

Question 73

What proportion of patients with sleep disordered breathing have PH

Answer 73

In obstructive sleep apnoea it is roughly 15-20% | If other sleep-related breathing disorders are included this rises to 17-53%

Question 74

Which paraneoplastic syndromes may be caused by squamous cell lung cancer

Answer 74

PTH/ PTHRP/ prostaglandin E production can lead to hypercalcaemia

Question 75

How might diffuse alveolar haemorrhage appear on CT 2-3 days after the haemorrhage

Answer 75

In between chronic bleeds Centrilobar nodules that are diffusely distributed Repeated haemorrhage may progress to fibrosis

Question 76

What causes Group 4 PH

Answer 76

Obstruction in the pulmonary arteries | Major cause is Chronic thromboembolic pulmonary hypertension (CTEPH

Question 77

Is there a gender difference in COPD

Answer 77

Previously more common in men However, more women in high income countries smoke and in low income women are at higher risk of indoor pollutants (cooking) so now equal amongst prevalence the sexes

Question 78

Which parts of the lung are most commonly affected by asbestosis

Answer 78

Begins in lower lobes & sub-pleura | Middle and upper become affected as it progresses

Question 79

How do you treat bronchiectasis

Answer 79

``` Antibiotics Mucus thinners - often nebulized Airway Clearance Devices Chest physio O2 therapy Quit smoking and avoid secondhand smoke Healthy lifestyle ```

Question 80

List the types of granulomatous diffuse parenchymal lung disease

Answer 80

Sarcoidosis | Hypersensitivity pneumonitis

Question 81

What is the definition of pneumoconiosis

Answer 81

Non-neoplastic lung reaction to inhalation of dusts encountered in the workplace; now includes chemical fumes & vapour inhalation too.

Question 82

How does asbestos cause asbestosis

Answer 82

Inhaled fibres settle at alveolar duct bifurcations Phagocytosis by macrophages triggers cytokine release Begins process of fibrosis

Question 83

In COPD, when the emphysema is mild, what is the main mechanism of airflow limitation

Answer 83

Bronchiolar abnormalities are most responsible for the majority of the deficit in lung function.

Question 84

The drugs given to treat the heart failure in cor pulmonale can have what complications

Answer 84

Diuretics – hypokaleamia and arrhythmia | Ace inhibitor – angioneurotic oedema

Question 85

When does CTEPH typically present

Answer 85

Typical age of onset is 60s.

Question 86

Describe the late phase reaction of atopic asthma

Answer 86

Consists of the recruitment of leukocytes (eosinophils, neutrophils and more T cells)

Question 87

What environmental factors can cause lung cancer

Answer 87

Tobacco Asbestos Radiation

Question 88

Describe the pathogenesis of bronchiectasis

Answer 88

Obstruction or infection result from a defect in airway clearance Specific causes - Primary ciliary dyskinesia and allergic bronchopulmonary aspergillosis

Question 89

In COPD what are the main clinical differences that will be seen in history and examination

Answer 89

COPD patient will typically have: Smoking history, older age, cough with sputum, slowly progressive exertional dyspnoea with less day-to-day variability; examination not usually abnormal until airflow limitation is severe

Question 90

Describe the pathogenesis of non-eosinophilic asthma

Answer 90

Mechanism is poorly understood | Some patients exhibit neutrophil-predominant disease with release of cytokines from T helper 1 cells,

Question 91

Which lung cancers could be classed as M1

Answer 91

M1a – Separate tumour nodule in contralateral lobe OR pleural nodules OR malignant or pericardial effusion M1b – Single extra-thoracic metastasis in a single organ M1c – Multiple extra-thoracic metastases

Question 92

Which population group is commonly affected by pulmonary haemorrhage?

Answer 92

Children, particularly premature or low weight infants

Question 93

List the symptoms of cor pulmonale

Answer 93

``` Dyspnea on exertion Fatigue Lethargy Exertional syncope and chest pain Abdominal distension Lower extremity swelling ```

Question 94

Which part of the lung is most commonly affected by idiopathic interstitial pneumonia

Answer 94

Lower zone, subpleural, and para-septal

Question 95

How does interstitial lung disease typically present on spirometry

Answer 95

Classical restrictive functional abnormalities | Reduction in diffusion capacity, lung volume and lung compliance.

Question 96

What is anaplasia

Answer 96

A lack of differentiation | Considered a hallmark of malignancy - they are highly anaplastic

Question 97

What is the most prevalent occupational disease in the world

Answer 97

Silicosis

Question 98

Describe the general pathogenesis behind diffuse parenchymal lung disease

Answer 98

Thought to begin with an acute injury to the pulmonary parenchyma This leads to chronic interstitial inflammation This results in -> fibroblast activation and proliferation with eventual pulmonary fibrosis + tissue destruction

Question 99

Describe the pathological features of subacute phase hypersentivity pneumonitis

Answer 99

Interstitial pneumonitis and non-caseating granulomas | Characteristically centred on bronchioles.

Question 100

Hypertrophic pulmonary osteoarthropathy is associated with what clinical sign

Answer 100

Finger clubbing

Question 101

What is the difference between tumour giant cells and inflammatory/foreign body giant cells

Answer 101

The inflammatory/foreign body giant cells are derived from macrophages and contain many small, normal-appearing nuclei The tumour giant cells have either a single huge polymorphic nucleus/two or more large hyperchromatic nuclei

Question 102

What is the underlying pathology in COPD

Answer 102

Chronic bronchitis and emphysema

Question 103

Small cell lung carcinoma is seen in non-smokers - true or false

Answer 103

False | 99% of cases occur in smokers

Question 104

Which paraneoplastic syndromes may be caused by small cell lung cancer

Answer 104

SIADH - leads to hyponatraemia Ectopic ACTH production leads to cushing syndrome

Question 105

How do you treat squamous cell lung cancer

Answer 105

Surgery, radiotherapy, chemotherapy Research ongoing into immunotherapy and angiogenesis inhibitors

Question 106

Which diseases affecting the lung vasculature can cause cor pulmonale

Answer 106

``` PE - large or recurrent Vasculitis Primary pulmonary hypertension ARDS Sickle cell disease ```

Question 107

Why does pulmonary vasoconstriction occur in response to hypoxia

Answer 107

Aims to increase ventilation/perfusion matching by diverting blood away from hypoxic areas Usually reverses when lung disease resolves - e.g. infection However, if hypoxia is chronic it can lead to PH

Question 108

How do you diagnose CTEPH

Answer 108

Chest radiography Echo - used to exclude other heart diseases V/Q scan - recommended first line if CTEPH suspected (can also exclude CTEPH) CT or MRI Catheter used to confirm PH

Question 109

What is emphysema

Answer 109

Defined pathologically as an abnormal, permanent enlargement of the air spaces distal to the terminal bronchioles, accompanied by destruction of their walls and without obvious fibrosis

Question 110

When does non-allergic asthma present

Answer 110

Can present at any age including during viral respiratory infections In children it is more likely to resolve in adolescence More common in adult women - especially if obese

Question 111

In which lung disease would you see bilateral hilar lymphadenopathy, erythema nodosum, fatigue and weight loss

Answer 111

Sarcoidosis

Question 112

List the pathological changes seen in the acute exudative phase of ARDS

Answer 112

Lungs are heavy, boggy, firm and red in colour There is oedema in the interstitium and alveoli alongside inflammation, deposition of fibrin and diffuse alveolar damage There are waxy hyaline membranes made from oedema fluid full of fibrin and parts of necrotic epithelial cells lining the alveoli

Question 113

What is desmoplasia

Answer 113

Growth of fibrous or connective tissues | Parenchymal cells of tumours can stimulate the formation of abundant collagenous stroma

Question 114

List the cytologic features of a malignant neoplasia

Answer 114

High nuclear to cytoplasmic ratio Pleomorphisms - variation in nuclear or cell size Hyperchromatic nuclei Mitoses present - especially irregular or bizarre Marked architectural disturbance and a loss of polarity

Question 115

What is the most common non-cutaneous cancer worldwide

Answer 115

Lung cancer

Question 116

List the microscopic features of Group 3 PH

Answer 116

Again depends on underlying lung disease Will also have the plexiform lesions seen in other PH groups Similar appearance to group 1 or ‘primary’ PAH

Question 117

What can cause diffuse pulmonary haemorrhage

Answer 117

Pulmonary vasculitis's (e.g. GPA, microscopic polyangiitis, Churg-Strauss and SLE) Goodpasture syndrome - non-ANCA vasculitis Can occur following a bone marrow transplantation All-trans-retinoic acid (ATRA) syndrome Pulmonary hemosiderosis – iron deposition in the lung Coagulative disorders Widespread metastasis – rare cause May be idiopathic

Question 118

What causes the pneumonia, abscess and/or lobar collapse seen in lung cancer

Answer 118

Airway obstruction

Question 119

What are the common symptoms of COPD

Answer 119

Cough, usually worse in the mornings and productive with a small amount of colourless sputum Breathlessness - most significant symptom, but usually does not occur until later on in disease Wheezing: particularly during exertion and exacerbations

Question 120

What do smokers macrophages look like

Answer 120

Abundant cytoplasm containing dusty brown pigment | Some macrophages have lamellar bodies (contain surfactant) within phagocytic vacuoles

Question 121

List the macroscopic features of Group 4 PH (CTEPH)

Answer 121

Yellow clots that contain collagen, fibroblasts, elastin, inflammatory cells and recanalization vessels. Stenosis and occlusion where the original PE was, usually with webs and bands. Vascular remodelling including hypertrophy and intimal thickening.

Question 122

How does activation of the inflammatory response affect the development of dust-borne diseases

Answer 122

Innate immune response increases intensity & duration of local reaction It is triggered by the phagocytosis of certain particles by macrophages

Question 123

Describe the differentiation of malignant neoplasia's

Answer 123

Poorly-differentiated | Exhibiting little or no evidence of differentiation

Question 124

Describe the blood supply to growing tumour cells

Answer 124

They must have a supply in order to grow However there is insufficient vascular stroma The tumour can develop areas of ischemia necrosis as a result

Question 125

Describe the trends in asthma mortality

Answer 125

There has been worldwide reduction in asthma mortality in adults and children over the past 25 years due to introduction of effective treatment (particualrly inhaled steroids) However, there is major disparity between countries Still causes may deaths - 417,918 deaths globally in 2016

Question 126

What is the survival rate in Group 1 PAH

Answer 126

Life threatening if not treated 5-year survival rate of 57% without treatment This drops to 1 year if they have right sided heart failure

Question 127

What are asbestos bodies

Answer 127

Long, thin asbestos fibres with translucent centre and gold-brown colour

Question 128

Describe the loss of polarity seen in malignant tumours

Answer 128

The orientation of anaplastic cells with respect to each other or to supporting structures like basement membranes (their polarity) is markedly disturbed Sheets or large masses of tumor cells grow in a disorganized fashion

Question 129

List potential complications of respiratory failure which are seen in the kidneys

Answer 129

Acute renal failure which can cause electrolyte and acid base disturbance

Question 130

What is dysplasia

Answer 130

Disordered growth

Question 131

Where does small cell lung carcinoma typically occur

Answer 131

Most commonly occur centrally in major bronchi, but can also be more peripheral

Question 132

Describe the presentation of subacute fibrotic lung diseases

Answer 132

Have a resolving, remitting, relapsing or progressive course Presentations with a variable course are typified by COP (weeks/months of prodromal flu symptoms; SOB + exercise intolerance). Can recur when steroids are withdrawn/tapered

Question 133

In situ epithelial cancers display all the cytologic features of malignancy - true or false

Answer 133

True | If not treated they have a high probability of progressing to invasive cancer

Question 134

How might diffuse alveolar haemorrhage appear on CXR

Answer 134

May vary based on underlying cause Diffuse infiltrative opacification pattern Typically in the mid zones with apical sparing

Question 135

Define each M stage in the TMN staging system (general cancer)

Answer 135

M0- no distant metastasis M1 - metastases present

Question 136

Define type 1 respiratory failure

Answer 136

PaO2 < 8kPa | Normal or low CO2

Question 137

What causes honeycomb fibrosis

Answer 137

Seen in IPF Destruction of the alveolar architecture This leads to the formation of cystic spaces lined by hyperplastic type II pneumocyte or bronchiolar epithelium

Question 138

Describe the differentiation of benign neoplasia's

Answer 138

They are well-differentiated Rare mitoses, of normal configuration e.g. lipoma only the growth is abnormal

Question 139

What is the most common type of pulmonary hypertension?

Answer 139

Group 2 PH caused by left-sided heart failure

Question 140

What are the 3 main mechanisms behind respiratory failure (generally)

Answer 140

V/Q mismatch Hypoventilation Shunting

Question 141

A person with CTEPH will likely have what in their clinical history

Answer 141

History of PE and/or the relevant risk factors for both PE and CTEPH

Question 142

Describe lepidic lung adenocarcinoma

Answer 142

Pneumocyte type neoplastic cells track along normal alveolar structure

Question 143

Why are patients with emphysema called pink puffers

Answer 143

Patients develop muscle wasting and weight loss Due to low cardiac output and hypoxia Will be breathing fast

Question 144

What is the most common form of asthma

Answer 144

Atopic or allergic asthma | Presen

Question 145

List the macroscopic features of asthma

Answer 145

Oedema Congestion Bronchial stenosis Mucus hypersecretion Seen in peripheral airways via scope

Question 146

How can you group fibrotic lung diseases

Answer 146

Chronic Subacute Acute

Question 147

List examples of chest wall disorders

Answer 147

Severe obesity Pleural diseases (e.g. trapped lung, scarring, large pleural effusions, chronic empyema) Kyphoscoliosis Neuromuscular diseases (e.g. MG, ALS, myopathy)

Question 148

How can COPD lead to Group 3 PH

Answer 148

Causes prolonged hypoxic vasoconstriction, mechanical stress, inflammation and endothelial dysfunction All contribute to increased pressure

Question 149

Which lung cancers could be classed as T4

Answer 149

Tumour > 7cm OR Involvement of the mediastinum, heart, great vessels, trachea, oesophagus, recurrent laryngeal nerve, vertebral body or carina OR Separate tumour nodules in a different ipsilateral lobe

Question 150

What is meant by the terms: tumour, mass or lesion

Answer 150

A growth or enlargement which may not be neoplastic (e.g. a granuloma)

Question 151

Do benign neoplasms spread

Answer 151

No

Question 152

Who is most affected by pulmonary haemorrhage

Answer 152

Children more than adults Appears to affect children of all ages Particualrly dangerous in premature babies or those with a low birth weight (high mortality in this group)

Question 153

How do you treat pulmonary haemorrhage

Answer 153

It depends on the exact underlying cause and the severity. Very premature babies or critically ill patients may require ventilation. Blood transfusion if the bleed is severe. Underlying disease managed as per individual guidelines. (e.g. steroids for CTD, Goodpasture's etc) If bleeding is coming from one site, it may be surgically embolised (blocked).

Question 154

How do you manage the heart failure in cor pulmonale

Answer 154

``` Salt restriction Diuretics Positive inotropes Ace inhibitors Cardiac resynchronisation therapy and mechanical ventricular assist devices ```

Question 155

Describe the general pathogenesis of COPD

Answer 155

Pathologic mechanisms not clear cut – few hypotheses Abnormal inflammatory response to inhaled particles Changes occur in central and peripheral airways and parenchyma This leads to airflow limitation

Question 156

List factors which increases your risk of developing CTEPH following a PE

Answer 156

Recurrent PE, large perfusion defects, high PA pressure at time of PE or an idiopathic PE Non-O blood group - 77% of CTEPH patients Splenectomy Hypothyroidism Both activate platelets ``` Ventriculoarterial shunt Infected intravenous catheters/devices Chronic inflammatory disorders Malignancy Abnormalities in blood components ```

Question 157

Is there a gender difference in asthma

Answer 157

YES Among children, asthma prevalence is higher in boys (due to smaller airways relative to lung size) However, it switches during adolescence with prevalence being around 20% higher in women than men (same reason reverses)

Question 158

How are tumours classified

Answer 158

Based primarily on the parenchymal component of the tumour itself

Question 159

List 'other' diseases that can cause cor pulmonale (i.e. not lung pathology)

Answer 159

``` Cerebrovascular diseases Obstructive sleep apnoea Scleroderma CF Obesity associated hypoventilation ```

Question 160

List potential complications of respiratory failure which are seen in the lungs

Answer 160

``` Pulmonary Fibrosis Pneumothorax Nosocomial pneumonia Ventilator dependency PE - due to acute RF ```

Question 161

How do you perform a pulmonary thromboarterectomy

Answer 161

Median sternotomy is performed Patient is put into hypothermic circulatory arrest for the surgery The thrombi are then dissected out of the vessels.

Question 162

What is pleomorphism

Answer 162

Variation in cell size and shape | Ranges from small cells within an undifferentiated appearance to tumor giant cells

Question 163

What are the 3 main subtypes of pulmonary haemorrhage

Answer 163

Diffuse pulmonary haemorrhage - diffuse bleeding into lung Diffuse alveolar haemorrhage - if the bleeding is into the alveolar spaces Localised pulmonary haemorrhage - haemorrhage is restricted to a focal region of the lung This can range from a small focus to a whole lobe

Question 164

Which features of respiratory failure suggest a poor prognosis

Answer 164

ARDS or COPD being the underlying cause Type 2 respiratory failure as patients tends to have chronic lung disease and renal, hepatic, neurological and cardiopulmonary co morbidities

Question 165

How many groups of pulmonary hypertension are there (as defined by the WHO)?

Answer 165

5 | 6 if you include the paediatric subtype but not an official group

Question 166

What increases risk of parenchymal lung disease

Answer 166

Exposure to dust, metals, organic solvents, and agricultural jobs. 

Question 167

List the microscopic features of emphysema

Answer 167

Abnormal large alveoli separated by thin septa with focal centriacinar fibrosis Loss of alveolar walls and dilatation of airspaces

Question 168

What features may be seen in dysplastic epithelial surfaces

Answer 168

Architectural disarray | A loss of orderly differentiation

Question 169

List the macroscopic features of Group 3 PH

Answer 169

Depends on the underlying lung disease | E.g., hyperplasia of mucus glands and emphysema seen in COPD

Question 170

What causes non-atopic asthma

Answer 170

Triggers are less clear | Possibly viral infection or air pollutants

Question 171

What causes the haemoptysis seen in lung cancer

Answer 171

Haemorrhage into the airways

Question 172

What factors suggest a poor prognosis in Group 1 PAH

Answer 172

Male sex Age over 50 Right ventricle dysfunction Poor functional status (as defined by WHO)

Question 173

Describe the structure of the granulomas that occur in sarcoidosis

Answer 173

They are well-formed non-necrotising granulomas containing aggregates of tightly clustered epithelioid macrophages (often with giant cells) In chronic cases the granulomas become enclosed within fibrous rims or may eventually be replaced by hyaline fibrous scars.

Question 174

What is the typical survival rate in IPF

Answer 174

Median survival 3.8 years after diagnosis | Diseases most similar to IPF have a mortality rate of approx. 50% at 5 years

Question 175

How might diffuse pulmonary haemorrhage appear on CT

Answer 175

Ground glass opacification +/- crazy paving pattern (ground-glass opacity with superimposed interlobular septal thickening and intralobular septal thickening) May have areas of consolidation Diffuse nodular densities on HRCT in the subacute phase

Question 176

How does CTEPH present on CT/MRI

Answer 176

Presence of thrombi and occlusion | Enlarged vessels

Question 177

List the characteristics of a benign tumour

Answer 177

Not harmful and not cancer - rarely fatal Localized at their site of origin Does not invade nearby tissue or spread to other parts of body (no mets) Slow growing Well differentiated - often resemble origin tissue Well circumscribed and encapsulated Generally amenable to surgical removal

Question 178

What causes the dyspnoea seen in respiratory failure

Answer 178

The excessive respiratory effort, hypoxia and hypercapnia affect the vagal receptors

Question 179

What is the common mechanism of death from asthma

Answer 179

Asthmatics are at increased risk of respiratory failure - occurs when not enough oxygen is transferred to blood Seen after life-threatening asthma attacks If respiratory failure isn’t treated immediately, it can lead to death Asthma patients might be at a greater risk of suffering cardiovascular and cerebrovascular diseases

Question 180

How does hypersensitivity pneumonitis present on pulmonary function tests

Answer 180

Acute restrictive pattern of lung function tests Symptoms occur 4-6hrs after exposure, can last 12hrs-several days. Will happen again re-exposure to antigen

Question 181

List the common inhaler therapies used in COPD

Answer 181

Start with a short acting bronchodilator as required Double therapy refers to a combination of LAMA, LABA and ICS (LAMA/LABA for those without asthmatic features and LABA/ICS for those with) Then move to triple therapy - LAMA/LABA/ICS

Question 182

What microscopic feature may be seen in acute exacerbations of IPF

Answer 182

Diffuse alveolar damage | May be super-imposed on the chronic changes

Question 183

Which PAH patients are treated with vasodilators

Answer 183

Group 1 PAH | Given to those who respond to the vasodilator reactivity tests

Question 184

Why must a pathologist be informed of the location of a biopsy sample

Answer 184

So that they can ensure it is the correct cell type for that location E.g. A sample of gastric mucosa is normal for the stomach but if it's from the bottom of the oesophagus its suggestive of metaplasia and comes with risks

Question 185

List the pathological changes seen in the proliferative phase of ARDS

Answer 185

Granulation tissue will form in the alveoli and their walls Type 2 pneumocytes will proliferate The granulation tissue can then either resolve causing little functional issues or it can cause fibrotic scarring in the alveolar septae This becomes the late fibrotic stage of ARDS

Question 186

What name is given to malignant tumours arising in solid mesenchymal tissues

Answer 186

Sarcomas

Question 187

The pathological findings of cor pulmonale are the same as which other condition

Answer 187

Right heart failure caused by left heart failure

Question 188

In which disease would you see Schaumann bodies

Answer 188

Sarcoidosis - characteristic | Also seen in other granulomatous diseases like TB

Question 189

How does smoking affect the development of dust-borne diseases

Answer 189

It worsens effects of all inhaled mineral dusts, but particularly those due to asbestos

Question 190

If the pulmonary artery becomes enlarged due to PAH what symptom can it cause

Answer 190

It can cause hoarseness if it compresses the laryngeal nerve

Question 191

List the macroscopic features of emphysema

Answer 191

Voluminous lungs - overlap the heart Upper 2/3 of lung most affected Alveoli can be seen on the cut surface of lung Apical blebs and bullae are indicative of severe disease

Question 192

List some potential complications of lung cancer

Answer 192

SVC syndrome Horner's syndrome Paraneoplastic syndromes Lambert-Eaton Syndrome

Question 193

What is the main cause of Group 1 PAH

Answer 193

Worldwide it is schistosomiasis - around 7% of sufferers develop it In Europe most cases are idiopathic as Schis not endemic there

Question 194

Which CTEPH patients are most likely to get surgery

Answer 194

It is based on underlying disease and where their clots are Larger proximal clots are ideal as easier to remove so more likely to get operated on Lots of small, distal clots may not get as much benefit from surgery Also depends on patient fitness

Question 195

List the microscopic features of asthma

Answer 195

Airway remodelling (detail in another card) Curschman spirals in sputum or bronchoalveolar lavage specimens Numerous eosinophils and Charcot-Leyden crystals- look like growing crystals

Question 196

Discuss the prevalence of COPD

Answer 196

The Global Burden of Disease Study reports a prevalence of 251 million cases globally in 2016. COPD is likely to increase in coming years due to higher smoking prevalence and aging populations in many countries

Question 197

List the causes of non-idiopathic interstitial pulmonary fibrosis

Answer 197

``` Occupational exposure (asbestosis, silicosis, etc.) Environmental antigens (farmer’s and pigeon-fancier lung, etc.) Drugs ± radiation Systemic illness (Hep C, HIV, IBD) ```

Question 198

What is hypersensitivity pneumonitis

Answer 198

A spectrum of immunologically mediated, mostly interstitial lung disorders, caused by intense, often prolonged, exposure to inhaled organic antigens

Question 199

What is CTEPH

Answer 199

Chronic thromboembolic pulmonary hypertension This is PH with organized, nonacute, thromboembolic material and altered vascular remodeling in the pulmonary vasculature It is a rare but dangerous complication of PE

Question 200

How does particle uptake by EC and travel across linings affect the development of dust-borne diseases

Answer 200

This allows direct interactions with fibroblasts & interstitial macrophages which can trigger immune responses Some particles may reach lymph nodes via lymphatic drainage directly or migrating macrophages triggering the adaptive immune response

Question 201

List some of the cause specific treatments used in respiratory failure

Answer 201

Bronchodilators in asthma Corticosteroids if airway swelling and inflammation Physical therapy and pulmonary rehab after the event especially if were on ventilator NG/parenteral feeding if ventilated

Question 202

How do lung diseases cause increased pulmonary hypertension

Answer 202

Most commonly cause hypoxemia chronically which leads to vasoconstriction and smooth muscle hypertrophy in the lungs vasculature as well as reduced NO Diseases of the pulmonary vasculature will cause PH COPD, bullous disease and PE’s will destroy capillary beds raising pressure COPD and mechanical ventilation will increase alveolar pressure causing PH

Question 203

Describe the histological signs of progressive silicosis

Answer 203

the early nodules coalesce into hard, collagenous scars (some soften and cavitate centrally due to superimposed TB or ischaemia) Hallmark histologically = central area of whorled collagen fibres w/ peripheral zone of dust laden macrophages. Nodules show weakly birefringent silicate particles on polarised microscopy

Question 204

List potential complications of non-invasive ventilation

Answer 204

Gastric aspiration can cause a pneumonia on top of an already diseased and struggling lung

Question 205

What are the characteristic features of asthma

Answer 205

Reversible airflow obstruction caused by bronchial hyperresponsiveness to a variety of stimuli as well as airway inflammation Chronic inflammation can lead to airway remodelling which is irreversible and causes permanent damage Variable and recurring symptoms

Question 206

COPD is most common in which patients

Answer 206

Long standing tobacco smokers Air pollutants may also contribute

Question 207

Is there geographical variation in COPD

Answer 207

More than 90% of COPD deaths occur in low­ and middle­ income countries

Question 208

List the microscopic features of complex coal worker's pneumoconiosis

Answer 208

Dense collagen and pigment | Has a centre of lesion often necrotic (local ischaemia)

Question 209

What is an adenoma

Answer 209

Benign neoplasm of the epithelial linings of ducts and glands

Question 210

List potential complications of invasive ventilation

Answer 210

``` Ventilator associated pneumonia Ventilator associated lung injury Pneumothorax Pulmonary oedema Hypoxemia due to lung damage ```

Question 211

There are no specific pathological findings for respiratory failure - true or false

Answer 211

True | The pathological findings would be those of the underlying condition causing the respiratory failure

Question 212

What is the most common primary lung cancer in never-smokers

Answer 212

Adenocarcinoma

Question 213

Metaplastic epithelium is prone to malignant transformation - true or false

Answer 213

True

Question 214

How does high altitude contribute to Group 3 PH

Answer 214

Long exposure causes hypoxia which in turn causes vasoconstriction

Question 215

How is cardiac catherisation used in PAH

Answer 215

Definitive diagnostic test | Also used to perform vasodilator tests

Question 216

How do you treat the underlying lung pathology in cor pulmonale

Answer 216

Each pathology will have its own specific treatment | Any that cause chronic hypoxia will get O2 therapy

Question 217

List the typical clinical features of Group 1 hypertension

Answer 217

Typically symptoms are related to the underlying cause ``` The PH itself can cause the following non-specific symptoms: Dyspnoea on exertion Fatigue Syncope (on exertion) Chest pain Anorexia RUQ pain ``` If it progresses to RHF you may see the associated congestive symptoms May also develop murmurs - splitting of second HS or systolic ejection

Question 218

List disorders affecting the neural drive of respiration that can be the underlying cause of respiratory failure

Answer 218

Drugs can depress it - narcotic and sedative overdose Structural changes - tumours/vascular abnormalities in the brainstem Metabolic abnormalities - myxoedema and chronic metabolic alkalosis

Question 219

Why might an occupational lung disease patient get an autopsy

Answer 219

They are often reported to fiscal so get one | You can get compensation for certain occupational diseases such as asbestosis

Question 220

Describe the features of carcinoma in situ

Answer 220

Severe dysplasia Involvement of full thickness of epithelium No penetration of basement membrane Irreversible

Question 221

What is the FEV1

Answer 221

Forced expiratory volume at one second | How much can you exhale in one second

Question 222

List the macroscopic features of bronchiectasis

Answer 222

Affects lower lobes bilaterally | The dilated bronchi appear cystic and are filled with mucopurulent secretions

Question 223

The lung cancer risk from asbestos exposure can be further increased by which other factor

Answer 223

Smoking 5x risk in asbestos alone 55x higher risk if they are also a smoker

Question 224

How do you treat Group 2 PAH

Answer 224

Optimise blood pressure through medication and lifestyle. Patients can stay active but should avoid strenuous exercise Typical heart failure treatment applies such as diuretics (loop), ACE or ARBs and beta-blockers. Vasodilators haven't been proven to be effective in this group yet. If causes by valve disease you can get valve replacement/repair. Cardiac transplant may be considered if medication is not effective.

Question 225

What are the macroscopic features of IPF

Answer 225

Cobblestoned pleural surface due to retraction of scars along interlobular septa Cut surface will be firm with rubbery white areas of fibrosis Lower lobes, subpleural regions & interlobular septa, affected preferentially.

Question 226

List the common symptoms of hypersentivity pneumonitis

Answer 226

Will have an acute attack following exposure to antigen | Includes fever, SOB, cough, leucocytosis

Question 227

Describe the typical clinical course of chronic respiratory failure

Answer 227

Tends to be less evident than the acute form Lasts a long time Usually has no cure Requires lifelong management

Question 228

Describe the pathophysiology of cor pulmonale

Answer 228

Lung disease cause an increase in pulmonary vascular resistance and arterial pressures - pulmonary hypertension Afterload increases Right heart has to work harder to overcome the afterload This leads to hypertrophy and subsequent dilatation - leads to HF The strain on the right heart is worsened by chronic hypoxia causing polycythemia which increases the blood viscosity increasing the work of the heart again

Question 229

How might acute phase diffuse alveolar haemorrhage appear on CT

Answer 229

The acute phase ranges from lobar areas of ground glass opacification to consolidation. Dark bronchus sign – bronchi appear darker than the surrounding ground glass opacities

Question 230

What are the common physical signs of COPD

Answer 230

Tachypnea and respiratory distress with simple activities Use of accessory respiratory muscles and paradoxical indrawing of lower intercostal spaces (Hoover sign) Cyanosis Elevated jugular venous pulse (JVP) Peripheral oedema

Question 231

Which lung cancers could be classed as N2

Answer 231

Metastasis to ipsilateral mediastinal or subcarinal lymph nodes

Question 232

What feature of mitoses is suggestive of malignancy

Answer 232

Atypical, bizarre, mitotic figures

Question 233

What is Acute interstitial Pneumonia (AIP)

Answer 233

It is a viral prodrome syndrome It's idiopathic Excludes those with ARDS which has an identifiable cause and those with underlying fibrotic lung disease or systemic disorders

Question 234

List the different types of diffuse parenchymal lung disease

Answer 234

``` Idiopathic interstitial pneumonias Non-idiopathic interstitial pulmonary fibrosis Granulomatous disease PLCH Eosinophilic pneumonia ```

Question 235

How are malignant neoplasms named

Answer 235

Same as benign neoplasms + certain additions | Named after cell origin

Question 236

List the features of dysplasia

Answer 236

May exhibit considerable pleomorphism Reversible Contain large hyperchromatic nuclei with a high N:C ratio More abundant mitotic figures may be seen

Question 237

Undifferentiated cancers often contain many cells in mitosis - true or false

Answer 237

True | They usually have a high rate of proliferation

Question 238

What is the most common malignancy in those exposed to asbestos

Answer 238

``` Lung cancer (5x increased risk) Mesothelioma is actually very rare even with exposure ```

Question 239

What is the major complication of Group 2 PAH

Answer 239

The backpressure can lead onto RVF

Question 240

Which lung cancers could be classed as T1

Answer 240

Tumour ≤ 3cm without pleural or mainstem bronchus involvement

Question 241

What are the general symptoms of interstitial lung disease

Answer 241

Dyspnoea, tachypnoea, dry cough, end-inspiratory ‘velcro’ crackles Hypoxemia, cyanosis and clubbing can occur, but late stage in disease course

Question 242

What is the definition of pulmonary haemorrhage

Answer 242

The term can be used for any form of bleeding from the lung, respiratory tract or alveoli

Question 243

Describe shunting

Answer 243

Deoxygenated blood passes the alveoli without being oxygenated due to abnormalities in the alveoli It then mixes with oxygenated blood that’s come through ventilated alveoli which causes the hypoxemia Occurs when there is a disease of the alveoli themselves that prevents the gas exchange such as pulmonary oedema, pneumonia, pulmonary haemorrhage or atelectasis

Question 244

List the different types of idiopathic interstitial pneumonias

Answer 244

Idiopathic pulmonary fibrosis (IPF) - most common Non-specific interstitial pneumonia (NSIP) Cytogenic organising pneumonia (COP) Acute interstitial pneumonia (AIP) Desquamative interstitial pneumonia (DIP) Respiratory bronchiolitis-associated interstitial pneumonia Lymphoid interstitial pneumonia

Question 245

What causes silicosis

Answer 245

Inhalation of the proinflammatory crystalline silicon dioxide Also called silica

Question 246

How might diffuse alveolar haemorrhage appear on CT in between chronic bleeds

Answer 246

Intralobular lines and thickening on top of the ground glass May have the crazy paving pattern

Question 247

How do you treat Desquamative interstitial pneumonia (DIP) and Respiratory bronchiolitis-associated interstitial lung disease

Answer 247

DIP responds well to steroids | Both are helped by smoking cessation

Question 248

Describe the early histological signs of silicosis

Answer 248

Tiny, hardly palpable, discrete pale-black (if coal present) nodules in hilar lymph nodes & upper zones

Question 249

What is the most common symptom of cor pulmonale

Answer 249

Exertional dyspnoea

Question 250

How does particle solubility and cytotoxicity affect the development of dust-borne diseases

Answer 250

Linked to particle size Small particles made of harmful substances of high solubility = more likely to produce rapid-onset acute lung injury Larger = more likely to resist dissolution & persist within lung parenchyma for years – tend to evoke fibrosing collagenous pneumoconioses

Question 251

What is the main aim of treatment for cor pulmonale

Answer 251

Treat the underlying lung pathology and the heart failure | Variety of drugs used, O2 therapy, salt restriction and some surgical options

Question 252

List the microscopic features of chronic bronchitis

Answer 252

Chronic inflammation of airways seen as: Presence of lymphocytes and macrophages Thickening of bronchiolar wall due to smooth muscle hypertrophy Deposition of extracellular matrix in muscle layer Peribronchial fibrosis Goblet cell hyperplasia Enlargement of mucus secreting glands

Question 253

How can you treat Group 1 PAH

Answer 253

``` O2 therapy - can reduce mortality Diuretics (loop are 1st choice) Digoxin Anticoagulants Vasodilators are given to some Lung transplantation is reserved for patients with severe cases ```

Question 254

What is a meningioma

Answer 254

A benign neoplasm of brain coverings

Question 255

List the histological features of Cryptogenic Organising Pneumonia (COP)

Answer 255

Polyploid plugs of loose organising CT (Masson bodies) within alveolar ducts, alveoli & bronchioles Connective tissue all the same age and underlying lung normal No interstitial fibrosis or honeycombing

Question 256

Which part of the lung is most affected in COPD

Answer 256

upper two thirds most commonly affected due to smoking (smoke rises)

Question 257

How does particle size affect the development of dust-borne diseases

Answer 257

Smaller particles can reach terminal small airways, air sacs and deposit in linings Most dangerous particles are 1-5micrometre in diameter

Question 258

How do you diagnose Group 1 PAH

Answer 258

Diagnosis of exclusion ECG and chest radiographs - may show ventricular dysfunction which suggests PAH A trans-thoracic echo with Doppler - used to measure PA pressure and assess ventricles. Definitive test is right-sided cardiac catherisation Further tests dependent on underlying cause

Question 259

What is the mechanism of death in Group 1 PAH

Answer 259

Most common cause of death is RHF (can lead to sudden cardiac death or arrhythmias) Dilation of the PA can cause dissection/rupture and compression of the left coronary artery.

Question 260

Describe the trend in the prevalence of lung cancer

Answer 260

Has been decreasing for both men and women since the 1950s

Question 261

List the different histological subtypes of lung adenocarcinoma

Answer 261

``` Lepidic Acinar Papillary Micropapillary Solid ```

Question 262

Describe an invasive carcinoma

Answer 262

Affects the surrounding tissues | Penetrate the basement membrane

Question 263

List the common symptoms of lung cancer

Answer 263

``` Weight loss Fever Cough Haemoptysis Chest pain Pneumonia, abscess, lobar collapse Hoarseness Diaphragm paralysis ```

Question 264

How can CTEPH cause death

Answer 264

If untreated it can progress to right ventricular failure and/or severe hypoxemic respiratory failure. Respiratory failure may require ventilation. Can lead to cardiogenic shock and death. Death due to shock or the other complications of RHF

Question 265

Describe the onset of late-onset asthma

Answer 265

Occurs in those aged 12-65 It is more severe, and associated with a faster decline in lung function, particularly in patients with a smoking history Includes occupational asthma

Question 266

How does COVID-19 interstitial pneumonia affect lung function

Answer 266

It makes it abnormal May see restrictive abnormalities, reduced diffusion capacity, small airways obstruction The abnormalities seem to be related to acute, severe episodes with high levels of inflammatory markers

Question 267

Describe the pathogenesis behind sarcoidosis

Answer 267

Includes several immunologic abnormalities suggesting cell-mediated immune response to unidentified antigen Exact cause unknown Includes intra-alveolar & interstitial accumulation of CD4+ T cells Raised levels of T cell-derived Th1 cytokines High levels of cytokines favour recruitment of more T cells + monocytes which can lead to granuloma formation Impaired dendritic cell function

Question 268

How does COVID-19 interstitial pneumonia present on imaging

Answer 268

Patients who have respiratory deterioration show ground-glass opacities on chest CT Fibrotic abnormalities

Question 269

Which condition increases your genetic susceptibility to COPD

Answer 269

Alpha 1-antitrypsin deficiency This is a deficiency in an elastase which should protect the lungs from elastic breakdown Without it you get early onset emphysema

Question 270

How do you treat Group 3 PH

Answer 270

Treatment is directed at the underlying lung disease. Ideally treat this early to prevent progression to or of the pulmonary hypertension. CPAP can reduce pressures in those with OSA – potentially reverses PH. Inhaled vasodilators can be used in COPD patients to improve V/Q matching.

Question 271

In which lung disease would you see eggshell calcification

Answer 271

Silicosis

Question 272

Small cell lung carcinoma is highly malignant - true or false

Answer 272

True There is virtually no pre-invasive phase 2/3 present with distant metastases – median survival of 10 months

Question 273

How do you manage asthma

Answer 273

Start with SABA alone Then offer a low dose ICS as maintenance Then introduce a LTRA in addition to ICS and review response If asthma is uncontrolled on this then offer a LABA in combination with the ICS, and review LTRA treatment Some more targeted treatments are available for specific types of asthma such as monoclonal antibodies

Question 274

How does environment affect the prevalence of asthma

Answer 274

Has been shown to alter prevalence Migrants from low prevalence areas to high areas will initially have the lower prevalence compared to native people but the incidence rises to a similar proportion with increasing length of stay

Question 275

What pathological features may you find in the liver and portal system of someone with cor pulmonale

Answer 275

Congestive hepatomegaly Nutmeg liver – pericentrally red and congested, periportally normal and tan Splenomegaly and platelet sequestration GI tract congestion and oedema

Question 276

Squamous cell lung carcinoma metastasizes late - true or false

Answer 276

True | Can be associated with better outcomes as a result

Question 277

Describe the histological appearance of squamous cell lung cancer

Answer 277

'Cobblestone’ appearance of polygonal cells Tumours may have central cavitation May see keratinisation with a central pearl

Question 278

Describe the pathogenesis of chronic bronchitis

Answer 278

Initiated by exposure to noxious chemicals which leads on to: Mucus hypersecretion CFTR dysfunction- acquired by smoking, abnormal dehydrated mucus Chronic inflammation Infection

Question 279

What name is given to a malignant neoplasm of the blood vessels

Answer 279

Angiosarcoma

Question 280

What is implied by the use of the term cancer

Answer 280

This implies malignancy

Question 281

What is anthracnosis

Answer 281

Form of pneumoconiosis Most innocuous coal-induced lesion Seen to some extent in city living & smokers)

Question 282

List the microscopic features of Group 1 PAH

Answer 282

Endothelial dysfunction. Decreased vasodilator production (prostacyclin, NO) and an increase in vasoconstrictors (endothelin). Plexiform lesions

Question 283

What causes the Curschman spirals seen in asthma

Answer 283

They occur due to extrusion of mucus plugs from sub-epithelial mucous gland ducts or bronchioles Will be seen in sputum of bronchoalveolar lavage specimens - look like long thing spirals (string?)

Question 284

In which tissues/organs are carcinomas in situ commonly seen

Answer 284

Skin Breast Bladder Uterine cervix

Question 285

Through what mechanisms can respiratory failure be fatal

Answer 285

``` Multiorgan failure (heart, brain, kidneys) due to inadequate O2 Sepsis Pulmonary dysfunction can be enough on its own Due to complications of respiratory failure (arrhythmia, MI, heart failure, GI haemorrhage, AKI, brain injury and death, heart failure, endocarditis) ```

Question 286

Typically what colour is lung cancer

Answer 286

Yellowish/grey

Question 287

Describe the progression of asbestosis

Answer 287

It is rare <10y from exposure More common presents after 20-30y Disease can remain static or progressive to respiratory failure etc

Question 288

When does metaplasia occur

Answer 288

Always found in association with tissue damage, repair and regeneration The tissue type can change to one better suited to the local environment - e.g. Barret's oesophagus

Question 289

What is the TNM staging system

Answer 289

The system used to stage all cancers T refers to the tumour itself and ranges from Tis-T4 N refers to nodes and ranges from N0-N3 M refers to metastasis and ranges from M0-M1

Question 290

In which lung disease would you see whorled collagen fibres with peripheral zone of dust-laden macrophages

Answer 290

Silicosis

Question 291

What causes respiratory failure

Answer 291

It happens when gas exchange within the lungs is inefficient This causes hypoxia and potentially hypercapnia

Question 292

What proportion of IPF patients have PH

Answer 292

28-46%

Question 293

What are the 3 main categories of lung cancers

Answer 293

Small cell - 15% of cases Non-small cell Metastases

Question 294

How does the prevalence of asthma vary geographically

Answer 294

The prevalence is highest in developed countries and lowest in developing countries However, rates in developing countries may be underestimated and is increasing rapidly in as lifestyles become westernized and more urbanized Stable or decreasing rates in developed countries

Question 295

What is cor pulmonale

Answer 295

Right heart failure that is caused by a lung pathology | not left heart failure

Question 296

What are the 4 main subtypes of asthma

Answer 296

Atopic Non-atopic Drug induced Occupational

Question 297

How can you differentiate between an old and recent fibrotic lesion in IPF

Answer 297

Earliest lesions identified by exuberant proliferation of fibroblasts (foci) As time goes on these become more fibrotic and less cellular

Question 298

List the microscopic features of Group 2 PAH

Answer 298

Intimal fibrosis and myofibroblast proliferation | Increased pressure and endothelial changes cause a release of growth factors which increase elastin production

Question 299

When does allergic asthma usually present

Answer 299

Usually presents in childhood Commonly associated with eczema, rhinitis, or food allergy, a family history of asthma (atopy) Presents with wheezing and coughing with and between viral infections

Question 300

Which blood abnormalities increase your risk of developing CTEPH

Answer 300

Abnormal fibrinogen structure Elevated factor VIII, von Willebrand factor, or type 1 plasminogen activator inhibitor Elevated lipoproteins

Question 301

What pathological features may you find in the body cavity of someone with cor pulmonale

Answer 301

Pericardial and pleural effusions Ascites Caused by the increased pressure

Question 302

What is senescence

Answer 302

Deterioration with age / loss of a cell’s power of division and growth Linked to telomere loss

Question 303

What results would be expected on the spirometry of a patient with restrictive lung disease

Answer 303

``` Normal FEV1:FVC ratio - >70% expected Variable FEV1 Reduced FVC Total lung capacity is reduced Reduced residual volume Normal airway resistance ```

Question 304

What name is given to a malignant neoplasm of the brain coverings

Answer 304

Invasive Meningioma

Question 305

How does hypersensitivity pneumonitis present on CXR

Answer 305

Multinodular interstitial infiltrates

Question 306

Describe SVC syndrome

Answer 306

Obstruction of the superior vena cava leads to facial swelling, dyspnoea, cough and orthopnoea

Question 307

Describe the general pathogenesis behind diffuse parenchymal lung disease

Answer 307

Thought to begin with an acute injury to the pulmonary parenchyma This leads to chronic interstitial inflammation This results in -> fibroblast activation and proliferation with eventual pulmonary fibrosis + tissue destruction

Question 308

What is the most common cause of pulmonary haemorrhage

Answer 308

Pulmonary vasculitis

Question 309

Describe the epidemiology of group 1 PAH

Answer 309

Estimated 6.6-26 cases per million adults in Europe Women are more likely to be affected (65-80% predominance) Average age at diagnosis is 50s No clear race predominance

Question 310

How does CTEPH present on chest radiography

Answer 310

May be normal until disease progresses Right ventricular enlargement Areas of lung hypoperfusion or infarction (Westmark sign and Hampton hump)

Question 311

In which part of the lung does adenocarcinoma typically occur

Answer 311

Tend to occur peripherally

Question 312

What are the main mechanisms behind type 1 respiratory failure

Answer 312

Type 1 is caused by V/Q mismatch, hypoventilation and shunting

Question 313

What causes the hoarseness seen in lung cancer

Answer 313

Recurrent laryngeal nerve invasion

Question 314

List the common oral therapies used in COPD

Answer 314

Prophylactic antibiotics Theophylline Corticosteroids - keep dose as low as possible and long term use not usually recommended Mucolytics - if chronic sputum production Roflumilast - long-acting inhibitor of the enzyme phosphodiesterase-4.

Question 315

What causes Horner's syndrome

Answer 315

Caused by pancoast tumours at lung apices infiltrating the sympathetic ganglia

Question 316

Which occupational lung disease features pleural plaques

Answer 316

Asbestosis

Question 317

How do you name benign tumours of epithelial cells

Answer 317

Some based on the cell of origin | Others are based on their microscopic appearance/macroscopic architecture

Question 318

What are the clinical characteristics of emphysema

Answer 318

Patients may be very thin with a barrel chest Patients typically have little or no cough or expectoration Severe dyspnoea from early on Breathing may be assisted by pursed lips and use of accessory respiratory muscles; patients may adopt the tripod sitting position The chest may be hyper resonant, and wheezing may be heard Heart sounds are very distant

Question 319

Describe the epidemiology

Answer 319

The prevalence can’t be known because it is a syndrome not its own disease

Question 320

List the pathological changes seen in the late fibrtoic phase of ARDS

Answer 320

Fibrotic scarring in the alveolar septae

Question 321

List the types of granulomatous diffuse parenchymal lung disease

Answer 321

Sarcoidosis | Hypersensitivity pneumonitis

Question 322

What is obstructive lung disease

Answer 322

Refers to a group of diseases characterized by an increase in resistance to airflow due to diffuse, widespread airway disease Can affect any level of the respiratory tract

Question 323

What name is given to a benign neoplasm of the connective tissues (fibrous tissues)

Answer 323

Fibroma

Question 324

What are the histological features of Acute interstitial Pneumonia (AIP)

Answer 324

Diffuse alveolar damage with subsequent fibrosis | DAD cause lung to be firm and rubbery

Question 325

List potential complications and subsequent cause of death in diffuse parenchymal lung diseases (DPLDs)

Answer 325

``` Progressive respiratory failure Cor pulmonale Pulmonary embolism Pneumothorax Super-imposed infections, e.g. pneumonia, TB Bronchogenic carcinoma ```

Question 326

What are the 5 WHO groups of pulmonary hypertension

Answer 326

Group 1 - Pulmonary arterial hypertension (PAH) Group 2 - Pulmonary hypertension due to left-sided heart disease Group 3 - Pulmonary hypertension due to lung diseases and/or hypoxia Group 4 - Chronic thromboembolic pulmonary hypertension (CTEPH) Group 5 - Pulmonary hypertension with unclear or multifactorial aetiologies There is also a paediatric-specific category which includes persistent pulmonary hypertension of the newborn.

Question 327

What are the principles of management of respiratory failure

Answer 327

Treat the underlying condition AND Correct the hypoxaemia with O2 management However, care needs to be taken with O2 management in type 2 respiratory failure - reduces hypoxic drive

Question 328

What results would be expected on the spirometry of a patient with obstructive lung disease

Answer 328

``` Reduced FEV1:FVC ratio - <70% expected Reduced FEV1 Normal FVC Total lung capacity normal or increased Normal or increased residual volume ```

Question 329

Which conditions can cause subgroup 4 in Group 1 PAH (e.g. conditions which cause localised lesion in the arterioles)

Answer 329

CREST, congenital L-R shunts, portopulmonary hypertension, HIV associated PAH and schistosomiasis

Question 330

What are the most common causes of management complications in the treatment of respiratory failure

Answer 330

O2 therapy | Invasive ventilation

Question 331

Which diffuse alveolar lung diseases are associated with smoking?

Answer 331

DIP and RB-ILD | Both respond to cessation

Question 332

What is a squamous cell carcinoma

Answer 332

Malignant neoplasm of stratified squamous epithelium

Question 333

Which areas of the body most commonly present with sarcoidosis

Answer 333

Most common clinical presentation is bilateral hilar lymphadenopathy or parenchymal lung involvement. 2nd most common – eye and skin lesions.

Question 334

List the microscopic features of Group 4 PH (CTEPH)

Answer 334

Microthrombi | Plexiform lesions

Question 335

In which cells is dysplasia principally found

Answer 335

Epithelial cells

Question 336

Describe the structure of Schaumann bodies

Answer 336

Laminated concretions of Ca & proteins Asteroid bodies = stellate inclusions Both found within giant cells in 60% of non-necrotising granulomas

Question 337

List potential complications of respiratory failure which are seen in the heart

Answer 337

Heart failure Arrhythmia MI ``` In acute RF particularly: Reduced CO Hypotension Arrhythmia Endocarditis MI ```

Question 338

Give an example of a marker of disease activity in sarcoidosis

Answer 338

High levels of TNF in the bronchoalveolar fluid | It is releases at increased levels by activated alveolar macrophages

Question 339

How does squamous cell lung cancer form

Answer 339

Starts as squamous metaplasia in bronchial epithelium | This leads to carcinoma in situ

Question 340

Give examples of obstructive lung diseases

Answer 340

``` Cystic Fibrosis Bronchiectasis Asthma Bronchitis (chronic) Emphysema ``` Last 2 combine for COPD

Question 341

Describe the features of asbestosis

Answer 341

Get fibrosis around respiratory bronchioles and alveolar ducts (adjacent alveolar sacs + alveoli can be affected) Diffuse interstitial fibrosis Causes distorted architecture - enlarged airspaces enclosed by thick fibrous walls Eventually honeycombing occurs Similar pattern of fibrosis as UIP Diffuse pulmonary interstitial fibrosis with asbestos bodies

Question 342

Which disease presents with patchy submucosal infiltrate of lymphocytes & histocytes

Answer 342

Respiratory bronchiolitis-associated interstitial lung disease

Question 343

List the main symptoms of asthma

Answer 343

Wheezing Shortness of breath Chest tightness and non-productive cough All episodic

Question 344

How do you treat CTEPH

Answer 344

Pulmonary thromboarterectomy is the definitive surgical treatment If ineligible for the thromboarterectomy, then balloon angioplasty can be performed to improve flow and perfusion It is recommended that patients go on lifelong anticoagulation (warfarin is typical choice)

Question 345

What causes Group 3 PH

Answer 345

Other lung disease or hypoxia. This includes: - COPD - Interstitial Lung Disease (ILD) such as idiopathic pulmonary fibrosis. - Sleep-disordered breathing disorders like obstructive sleep apnoea. - Alveolar hypoventilation disorders - Long exposure to high altitude

Question 346

List common signs of cor pulmonale

Answer 346

``` Cyanosis Tachycardia Raised JVP Loud pan systolic murmur Ascites Peripheral oedema Hepatosplenomegaly ```

Question 347

What is the prevalence of asthma

Answer 347

It is one of the most common chronic, non-communicable diseases worldwide 4% of global adult population diagnosed

Question 348

List potential complications of respiratory failure which are seen in the brain

Answer 348

Irreversible brain damage and brain death

Question 349

Which neuromuscular diseases can cause cor pulmonale

Answer 349

Myasthenia gravis Poliomyelitis MND

Question 350

List the types of inflammation that asthma can be divided into

Answer 350

eosinophilic (allergic and non-allergic) Non-eosinophilic (neutrophilic type 1 and type 17 and paucigranulocytic) Mixed granulocytic inflammation

Question 351

What is the normal nucleus:cytoplasm ratio

Answer 351

Between 1:4 and 1:6

Question 352

List genetic changes that make you more susceptible to asthma

Answer 352

Chromosome 5q locus – near genes for IL-4, IL-5, IL-9, IL-13 and receptor for IL-4 Class 2 HLA alleles linked to production of IgE against some allergens Mutations of genes encoding IL-33 and its receptor result in production of TH2 cytokines Mutations of gene encoding TSLP which is a cytokine with a role in initiating allergic reactions

Question 353

What are the 2 main subgroups of group 4 PH

Answer 353

Cases caused by chronic thromboembolic pulmonary hypertension (CTEPH) Cases caused by other obstructive causes (e.g. tumours, congenital stenosis).

Question 354

Describe the appearance of nutmeg liver

Answer 354

Pericentrally red and congested | Periportally normal and tan

Question 355

What is squamous cell lung cancer associated with

Answer 355

Smoking

Question 356

What is another name for small cell lung carcinoma

Answer 356

oat cell carcinoma

Question 357

Describe the general prognosis for lung cancer

Answer 357

Generally poor as disease tends to be advanced when it presents Overall 5-year survival rate of 18.7%

Question 358

List potential complications of O2 therapy in respiratory failure

Answer 358

Patients with type 2 respiratory failure may use a hypoxic drive to drive their breathing (an effect of chronic hypercapnia) O2 therapy can reduce this hypoxia and therefore reduce their breathing drive

Question 359

What is the definition of differentiation (in regards to neoplasia)

Answer 359

The extent to which neoplastic parenchymal cells resemble the corresponding normal parenchymal cells morphologically and functionally

Question 360

How does interstitial lung disease typically present on CXR

Answer 360

Bilateral small nodules | Irregular lines or ground-glass shadows all corresponding to areas of interstitial fibrosis.

Question 361

Which lung cancers could be classed as M0

Answer 361

Those with no distant metastases

Question 362

What pathological features may you find in the heart of someone with cor pulmonale

Answer 362

Right heart hypertrophy and dilatation | Left heart will be unaffected

Question 363

Which intrinsic lung disease present in the younger age groups (20-40)

Answer 363

Sarcoidosis Collagen-vascular-associated diseases Pulmonary Langerhans cell histiocytosis

Question 364

Describe the mortality trends in Group 2 PAH

Answer 364

Group 2 has a reduced survival compared to other types of PH. Presence of PH in those with LHD leads to greater mortality.

Question 365

What are the main pathological findings of cor pulmonale

Answer 365

``` Pericardial and pleural effusions Ascites Hepatosplenomegaly Nutmeg liver Pretibial pedal and presacral oedema GI tract oedema Pathological findings of underlying cause Hypertrophy and dilatation confined to the right side of the heart ```

Question 366

Symptoms develop quickly in lung cancer - true or false

Answer 366

False | Usually insidious and non-specific leading to late diagnosis

Question 367

How may PAH appear on a CXR

Answer 367

Enlarged pulmonary arteries Reduction in blood vessels across the lung fields (oligemic) May have right heart dilatation

Question 368

What causes occupational asthma

Answer 368

Exposure to occupational antigens such as: Fumes (resins) Dusts (cotton) Gases (toluene), chemicals (formaldehyde)

Question 369

List the microscopic features of airway remodelling seen in asthma

Answer 369

Airway remodelling: Thickening of the airway wall Subbasement membrane fibrosis (deposition of type 1 and 3 collagen) Increased vascularity, Increase in the size of goblet cells Hypertrophy and/or hyperplasia of bronchial wall muscle Increased extracellular matrix

Question 370

What name is given to malignant tumours arising in epithelial cells

Answer 370

Carcinomas

Question 371

List potential nutritional complications of respiratory failure

Answer 371

Malnutrition Diarrhoea due to NG feeding Hypoglycaemia and electrolyte disturbance due to parenteral feeding

Question 372

List the common features of hypoxia

Answer 372

``` Dyspnea Restlessness Agitation Confusion Central cyanosis Tachycardia and arrhythmia Myoclonus Seizures ```

Question 373

Describe the epidemiology of cor pulmonale

Answer 373

It's exact prevalence is unknown In the U.S. 6-7% of adult heart disease is CP Countries with lots of smoking and air pollution will have higher prevalence's as they will have higher rates of lung disease

Question 374

Describe hypoventilation

Answer 374

The lungs are not being ventilated properly Gradients of PaO2, PaCO2 and intra alveolar O2 remain normal Instead caused by CNS damage and depression, chest wall and muscular issues

Question 375

Patients with Group 2 PAH may have a history of what other conditions

Answer 375

MI, cardiomyopathy, hypertension or pericarditis

Question 376

Explain how malignant neoplasms spread

Answer 376

``` Either by: Direct invasion of surrounding tissues Via the lymphatic system Via haematogenous spread Or by seeding ```

Question 377

Idiopathic pulmonary fibrosis is rare in children - true or false

Answer 377

True | Most patients with IPF are >50 y/o

Question 378

How does Group 3 PH progress

Answer 378

Increase in pulmonary pressure will eventually lead to heart failure

Question 379

What complications can arise when treating COPD patients with O2

Answer 379

COPD patients may have chronic hypercapnia and therefore use hypoxia for respiratory drive By giving them O2 you can remove this drive If its home O2 therapy need to ensure they don’t smoke to avoid explosion

Question 380

What is a haemangioma

Answer 380

A benign neoplasm of the blood vessels

Question 381

Describe the pathology behind hypersensitivity pneumonitis

Answer 381

There is an abnormal sensitivity/heightened reactivity to antigen which leads to pathological changes involving alveolar walls 2/3 develop non-necrotising granulomas which suggest T-cell mediated (IV) hypersensitivity reaction Can progress to severe chronic fibrotic lung disease if not addressed

Question 382

Is is chronic bronchitis or emphysema that is associated with infection

Answer 382

Chronic bronchitis | Infections occur occasionally in emphysema but much more common in CB

Question 383

List the potential pharmalogical treatments for diffuse parenchymal lung diseases (DPLDs)

Answer 383

Prednisolone ± cytotoxic agents (potential steroid-sparing effect) in some diagnoses. Antifibrotic mediations, e.g., colchicine, cyclosporine, may reduce the rate of decline in FVC in patents with IPF. In general, NSIP, DIP, & COP are more responsive to corticosteroids and immunosuppressives. UIP generally unresponsive to these. Tyrosine kinase inhibitors and TGF-b antagonists have been shown to slow progression of IPF and are the only targeted therapy so far.

Question 384

How does lung cancer incidence change with age

Answer 384

Incidence increases as age does Peaks for both men an women in 70s Starts decreasing for 80+

Question 385

What causes the airway remodelling seen in asthma

Answer 385

Repeated inflammation due to allergen exposure | This leads to irreversible damage

Question 386

Which is the most common idiopathic interstitial pneumonia?

Answer 386

IPF

Question 387

What is metaplasia

Answer 387

Replacement of one type of cell with another type

Question 388

What external pathological findings may be seen in a person with cor pulmonale

Answer 388

Pretibial and pedal oedema Presacral oedema in bed ridden patients Ansarca rarely (general swelling of whole body)

Question 389

How can you differentiate between asthma and COPD if history and exam leaves uncertainty

Answer 389

Asthma will have a large response (over 400ml) to bronchodilators and oral steroids (COPD wont) Serial peak flow measurements showing 20% or greater diurnal or day-to-day variability suggest asthma Clinically significant COPD is not present if the FEV1 and FEV1/FVC ratio return to normal with drug therapy.

Question 390

What are the clinical characteristics of chronic bronchitis

Answer 390

Patients may be obese Frequent cough and spitting are typical Use of accessory muscles of respiration is common though dyspnoea is milder Coarse rhonchi and wheezing may be heard on auscultation Patients may have signs of right heart failure (i.e. cor pulmonale), such as edema and cyanosis Infections are common

Question 391

What causes atopic asthma

Answer 391

Caused by a Th2 and IgE mediated response to environmental allergens Characterized by an immediate phase and a late phase reaction

Question 392

If the cor pulmonale is caused by CTEPH or PE what other treatment may be required

Answer 392

Anticoagulation

Question 393

How might localised pulmonary haemorrhage appear on CXR/CT

Answer 393

hazy consolidation or ground glass infiltrates

Question 394

What causes bronchiectasis

Answer 394

Congenital and hereditary conditions Severe necrotizing pneumonia Immune disorders Bronchial obstruction Idiopathic - 50% of cases

Question 395

Describe the pathogenesis of emphysema

Answer 395

Toxic injury and inflammation Protease-antiprotease imbalance (e.g. increase in protease = more breakdown) Oxidative stress - may enhance inflammatory response

Question 396

List the causes of non-idiopathic interstitial pulmonary fibrosis

Answer 396

``` Occupational exposure (asbestosis, silicosis, etc.) Environmental antigens (farmer’s and pigeon-fancier lung, etc.) Drugs ± radiation Systemic illness (Hep C, HIV, IBD) ```

Question 397

What is the aim of treatment in Group 2 PAH

Answer 397

Mainly involves treating the left-sided heart failure.

Question 398

Which lung cancers could be classed as N0

Answer 398

Those with no metastasis to regional lymph nodes

Question 399

The presence of mitoses means it is a malignancy - true or false

Answer 399

False | Can be seen in normal cells - e.g. dividing or hyperplastic

Question 400

How might diffuse pulmonary haemorrhage appear on CXR

Answer 400

Often non-specific | May see bilateral air-space consolidation with apical sparing

Question 401

What is the definition of neoplasia

Answer 401

New, uncontrolled growth of cells that is not under physiologic control

Question 402

Describe the characteristics of non-specific interstitial pneumonia

Answer 402

Cellular and fibrosing pattern. Fibrosis: diffuse/patchy interstitial fibrotic lesions of roughly the same age (important distinction from UIP) No fibroblastic foci, honeycombing, hyaline membranes or granulomas

Question 403

List the different types of idiopathic interstitial pneumonias

Answer 403

Idiopathic pulmonary fibrosis (IPF) - most common Non-specific interstitial pneumonia (NSIP) Cytogenic organising pneumonia (COP) Acute interstitial pneumonia (AIP) Desquamative interstitial pneumonia (DIP) Respiratory bronchiolitis-associated interstitial pneumonia Lymphoid interstitial pneumonia

Question 404

Which factors affect the development of dust-borne diseases

Answer 404

Dust retention - how much stays in lungs Particle size Particle solubility and cytotoxicity Particle uptake by EC or travel across the linings Activation of the inflammatory system Tobacco smoking

Question 405

List potential complications of respiratory failure which are seen in the GI tract

Answer 405

Stress ulcers Ileus Haemorrhage Gastric distension

Question 406

Define type 2 respiratory failure

Answer 406

PaO2 < 8 kPa PaCO2 > 6kPa High CO2

Question 407

List the potential mechanisms of death in cor pulmonale

Answer 407

CP can decompensate Rarely can progress to LHF Massive peripheral oedema can cause hypovolemic shock Hypertrophied cardiac tissue may become ischemic which can lead to arrhythmias Renal congestion can lead to azotemia (excess N) CP can cause venous congestion and hypoxia in the CNS Venous congestion and hypoxia in the CNS caused by CP can also be fatal Massive PE that caused the CP can lead to obstructive shock Complications of the underlying cause of CP can be fatal All of the above can be fatal

Question 408

Which fibrotic lung diseases come under the chronic category

Answer 408

Those resembling IPF and usually share a common pathology, e.g. UIP. Many of the rheumatologic/CTDs, chronic HSP, radiation and drug-related pulmonary fibrosis, occupational exposures.

Question 409

A malignant lesion on the R lung is approximately 4 cm across and is invading the main bronchus. Biopsies show involvement of the left hilar lymph nodes, and metastases have been found in the brain and spine. What is the correct staging using the TNM system?

Answer 409

T2 - Tumour 3-5cm and involving main bronchus N3 - mets to contralateral hilar nodes M1 - mets present

Question 410

What is a fibrosarcoma

Answer 410

Malignant neoplasm of the connective tissues (fibrous tissues)

Question 411

List examples of chronic interstitial and infiltrative disease

Answer 411

Pneumoconiosis Interstitial fibrosis DALDs

Question 412

What name is given to a malignant neoplasm of the epithelial linings of ducts and glands

Answer 412

Adenocarcinoma

Question 413

Give examples of vasodilator drugs

Answer 413

Endothelin receptor antagonists - ambrisentan) PDE-5 inhibitors – tadalafil Prostacyclin analogues or agonists – beraprost

Question 414

What is restrictive lung disease

Answer 414

Refers to diseases which involve reduced expansion of lung parenchyma and result in a decreased lung capacity

Question 415

What are the complications of CTEPH treatment

Answer 415

Aside from the typical risks of cardiothoracic surgery, there are 2 main complication that occur during thromboarterectomy: Pulmonary artery seal – the redistribution of blood flow causes a severe V/Q mismatch Reperfusion pulmonary oedema- fluid shift occurs in the surgical area Also have the typical side effects of medications.

Question 416

In COPD, when the emphysema is moderate or severe, what is the main mechanism of airflow limitation

Answer 416

The loss of elastic recoil caused by emphysema | Rather than the bronchiolar disease

Question 417

Describe the macroscopic features of sarcoidosis seen in the lungs

Answer 417

No macroscopic changes except in advanced cases where granulomas join to form small nodules These are palpable or visible as 1-2cm non-caseating, non-cavitated consolidations and are primarily distributed along lymphatics and around bronchi and blood vessels.

Question 418

Which lung cancers could be classed as T2

Answer 418

``` Tumour 3-5cm OR Involvement of the visceral pleura or mainstem bronchus but not the carina OR Lobar atelectasis ```

Question 419

Silicosis is rapidly fatal - true or false

Answer 419

False | It is a slow killer but significantly impaired lung function impacts QoL

Question 420

What is the most common form of idiopathic interstitial pneumonia

Answer 420

Interstitial pulmonary fibrosis (IPF)

Question 421

How do you manage Cryptogenic Organising Pneumonia (COP)

Answer 421

Some recover spontaneously Most need oral steroids for 6m+ for complete recovery Long-term prognosis depends on underlying disorder

Question 422

Describe the typical clinical course of acute respiratory failure

Answer 422

Comes on quickly Much more serious and life threatening than the chronic course Can be managed at the time and resolve or leaves long term complications

Question 423

List the potential surgical treatments for cor pulmonale

Answer 423

Venesection if hematocrit >65% in polycythemia Pulmonary embolectomy in PE that’s haemodynamically unstable Single/double lung and heart transplants in terminal stages in young patients

Question 424

What happens when mast cells degranulate in atopic asthma

Answer 424

They produce cytokines and other mediators which induce induce the early phase hypersensitivity reaction and then eventually the late phase reaction The mediators trigger the bronchoconstriction by stimulating the parasympathetic system

Question 425

What determines the rate of growth of a tumour

Answer 425

Doubling time of tumor cells Fraction of cells that have the ability to replicate The rate at which tumor cells die and shed

Question 426

Describe the histological features of small cell lung carcinoma

Answer 426

Poorly differentiated cells with little cytoplasm and ill-defined borders

Question 427

What is the most common cause of acute onset cor pulmonale

Answer 427

Massive PE

Question 428

What is the prognosis of cor pulmonale

Answer 428

50% deceased within 5 years | Prognosis depends on underlying cause with lung parenchymal disease having a worse prognosis

Question 429

Describe the appearance of the plexiform lesions seen in pulmonary hypertension

Answer 429

Lesions have medial hypertrophy, eccentric or concentric laminar intimal proliferation and fibrosis, fibrinoid degeneration, and thrombotic lesions

Question 430

List potential complications of tracheotomy

Answer 430

Haemorrhage Recurrent laryngeal and vagus nerve damage Pneumothorax Obstruction of the airway from mucus and secretions or from scarring in healing Collapse of the trachea

Question 431

Which drugs can induce PAH in Group 1 | e.g. subgroup 3

Answer 431

Aminorex, fenfluramine derivatives, and toxic rapeseed oil | Other risks included amphetamine, cocaine, St John's wort

Question 432

Which other diagnoses must be excluded when considering sarcoidosis

Answer 432

TB, fungal infections and berylliosis | All can also cause non-caseating granulomas

Question 433

List the subdivisions of group 1 PAH

Answer 433

Group 1 - Pulmonary arterial hypertension - idiopathic Group 2 - heritable PAH - caused by gene mutations included in BMPR2 and ALK2 Group 3 - drug or toxin induced Group 4 - due to conditions which cause localised lesion in the arterioles

Question 434

List the microscopic features of IPF

Answer 434

Patchy interstitial fibrosis which varies in intensity and age Typical to see both early and late lesions at same time Honeycomb fibrosis Mild to moderate inflammation in the fibrotic areas - mostly lymphocytes, few plasma cells, neutrophils, eosinophils & mast cells Foci of squamous metaplasia + SM hyperplasia may be present May also have pulmonary arterial hypertensive changes Diffuse alveolar damage in acute exacerbations

Question 435

List some of the effects that asbestos has on the lungs

Answer 435

``` Localised fibrous plaque Pleural effusions (recurrent) Parenchymal interstitial fibrosis (asbestosis) Lung carcinoma Mesothelioma ```

Question 436

Which left-sided heart diseases can cause Group 2 PAH

Answer 436

``` Systolic dysfunction Diastolic dysfunction Valvular defects Congenital or acquired inflow/outflow tract obstruction Congenital cardiomyopathies. ```

Question 437

What are the symptoms of Acute interstitial Pneumonia (AIP)

Answer 437

Sudden onset dyspnoea Rapid development of respiratory failure Non-productive cough

Question 438

Sarcoidosis lesions in the lungs have a tendency to heal - true or false

Answer 438

True | Often there is – varying stages of fibrosis and hyalinisation seen

Question 439

Which other diseases does asbestosis increase your risk of

Answer 439

5-fold increase in lung cancer - it is a tumour initiator + promoter If you also smoking = 55-fold increase in lung cancer Toxic chemicals such as tobacco carcinogens can attach to the asbestos fibres and make them a more potent carcinogen

Question 440

Radiographic imaging in patients with group 2 PAH may show what

Answer 440

``` Enlarged PA Ventricular dilatation Enlarged heart Oedema Dilated left atrium is a sign of chronic elevated left sided pressures ```

Question 441

What percentage of lung cancers are caused by tobacco

Answer 441

70%-90% of all cases

Question 442

How do you manage COPD

Answer 442

Provide with info and support Assess and address smoking – nicotine replacement therapy Vaccinations - Offer pneumococcal vaccination and an annual influenza Develop an individualized exacerbation action plan Pulmonary rehabilitation + chest physio Inhaled and oral therapies

Question 443

Describe the course of acute fibrotic lung diseases

Answer 443

Has a fulminant, progressive, remitting, or resolving course

Question 444

Describe the pathogenesis of Group 2 PAH

Answer 444

Left sided pressures increase and causes backpressure in the pulmonary venous system Persistent high pressures lead to structural and functional changes in the pulmonary vessels This includes vasoconstriction which increases the mean pulmonary arterial pressure. Right ventricular systolic pressure increases in order to maintain CO

Question 445

In patients with type 1 respiratory failure, what concentration of O2 should be given as treatment

Answer 445

24-60% O2

Question 446

What type of pleural effusion can be caused by asbestosis

Answer 446

Usually serous but can be bloody

Question 447

Describe the pathological features of acute phase hypersentivity pneumonitis

Answer 447

Acute alveolar damage (1st hrs-days from exposure)

Question 448

How can pulmonary haemorrhage lead to death

Answer 448

Repeated bleeds can lead onto organising pneumonia, collagen deposition in the vessels and eventually pulmonary fibrosis. All of this disruption can be fatal Haemorrhage related respiratory failure is a common cause of death

Question 449

How might localised pulmonary haemorrhage appear on CXR/CT

Answer 449

hazy consolidation or ground glass infiltrates

Question 450

How does squamous cell lung cancer present on radiographs

Answer 450

Asymptomatic and invisible

Question 451

Which part of the tumour determines its growth and spread

Answer 451

The stroma

Question 452

What are the main mechanisms behind type 2 respiratory failure

Answer 452

Type 2 is caused by alveolar hypoventilation with/without V/Q mismatch

Question 453

Define each T stage in the TMN staging system (general cancer)

Answer 453

Tis - carcinoma in situ T1 - Tumour ≤ 3cm T1a – ≤ 1cm T1b – 1-2cm T1c – 2-3cm T2 - Tumour 3-5cm T3 - Tumour 5-7cm T4 - Tumour > 7cm

Question 454

List the diseases that cause diffuse alveolar filling that can be the underlying cause of respiratory failure

Answer 454

Cardiogenic and neurogenic oedema Pneumonia Extensive pulmonary haemorrhage They all lead to inadequate gas exchange

Question 455

List disorders of the peripheral nervous system, respiratory muscles and chest wall that can be the underlying cause of respiratory failure

Answer 455

``` Guilliane Barre syndrome Myasthenia gravis Muscular dystrophy Morbid obesity Kyphoscoliosis ``` They all cause inadequate ventilation causing hypoxia and hypercapnia

Question 456

Tobacco smoking accounts for as much as 70% of COPD cases - true or false

Answer 456

False | Its as much as 90%

Question 457

What can cause hypersensitivity pneumonitis

Answer 457

Exposure to inhaled organic antigens | Includes: bird fancier’s lung, farmer’s lung, etc.

Question 458

List the macroscopic features of chronic bronchitis

Answer 458

Hyperemia Swelling Oedema of mucus membranes Heavy casts of secretions and pus fill the bronchi and bronchioles

Question 459

Which types of cancer can be caused by asbestos exposure

Answer 459

Lung carcinoma Mesothelioma Extra-pulmonary neoplasms such as laryngeal or ovarian

Question 460

What are the clinical features of respiratory failure

Answer 460

They will have clinical features of the underlying cause (e.g. pneumonia > fever, cough, sputum etc.) Dyspnoea is the common symptom Will have features of hypoxia and hypercapnia (other card)

Question 461

List diseases that can lead to Type 1 respiratory failure

Answer 461

``` COPD Pneumonia Asthma Pulmonary oedema Pulmonary fibrosis PE Pulmonary Hypertension ARDS ``` Many more

Question 462

Describe the pathological features of chronic phase hypersentivity pneumonitis

Answer 462

Interstitial fibrosis with fibrocytic foci Honeycombing Obliterative bronchitis Loosely-formed granulomas

Question 463

Describe the pathogenesis behind IPF

Answer 463

It appears to be a primary disorder of fibroblast activation + proliferation in response to an unknown trigger Genetic analyses point towards roles of deterioration of the alveolar epithelium due to telomere shortening, altered mucin production, and abnormal signaling in alveolar fibroblasts Injury to alveolar epithelial cells triggers increased local production of fibrogenic cytokines

Question 464

In which lung disease would you see weakly birefringent silicate particles

Answer 464

Silicosis

Question 465

What is the most common type of PAH

Answer 465

Group 2 | That caused by left heart disease

Question 466

What are the clinical features of Group 3 PH

Answer 466

Patient will have the symptoms of the underlying lung disease alongside the typical PH symptoms Again can go on to develop right-heart failure or cor-pulmonale and their associated symptoms

Question 467

Which lung cancers could be classed as T3

Answer 467

Tumour 5-7cm OR Involvement of the parietal pleura, chest wall, diaphragm, phrenic nerve, mediastinal pleura or parietal pericardium OR Separate tumour nodules in the same lobe

Question 468

How does silicosis present

Answer 468

SOB only when progressive massive fibrosis

Question 469

Describe the pathogenesis behind occupational lung diseases

Answer 469

Environmental trigger from occupational dusts etc. | However, only a small % of those exposed will develop the disease which implies there is also a genetic predisposition

Question 470

Describe the epidemiology of pulmonary haemorrhage

Answer 470

Affects only 1 in 1000 live births | More common in premature or low birth weight babies

Question 471

Describe the presentation of subacute fibrotic lung diseases

Answer 471

Presentations with a variable course are typified by COP (weeks/months of prodromal flu symptoms; SOB + exercise intolerance). Can recur when steroids are withdrawn/tapered

Question 472

What is the definition of pulmonary hypertension

Answer 472

Mean pulmonary arterial pressure of over 25mmHg at rest or over 30mmHg during exercise. Will also have a capillary wedge pressure of <15mmHg.

Question 473

Where are normal mitoses seen in the body

Answer 473

Seen in normal tissues exhibiting rapid turnover e.g. epithelial lining of gut Also seen in non-neoplastic proliferations like hyperplasia's

Question 474

How does ILD lead to Group 3 PH

Answer 474

Chronic injury to the alveolar membranes and fibrosis with vascular remodelling

Question 475

What are the clinical features of Group 2 PAH

Answer 475

Symptoms can be non-specific but are usually progressive Similar symptoms to group 1 but group 2 patients generally have increased dyspnea, lower exercise tolerance and shorter survival Again, it can progress to RHF (and therefore develop the common symptoms)

Question 476

What is lepidic adenocarcinoma also known as

Answer 476

Bronchoalveolar carcinoma | That's the old name

Question 477

How do you treat small cell lung carcinoma

Answer 477

Limited disease is sensitive to chemotherapy and radiotherapy but 5-year survival is only 10%

Question 478

List common symptoms of sarcoidosis

Answer 478

``` Insidious SOB Cough Chest pain Haemoptysis Constitutional symptoms Skin changes e.g., erythema nodosum, or uveitis might also suggest sarcoid ```

Question 479

What causes the pathological findings in cor pulmonale

Answer 479

The right-heart failure Systemic and portal venous congestion due to backpressure May have some findings from the underlying lung disease

Question 480

What is the only definitive therapy for IPF

Answer 480

Lung transplantation

Question 481

List the microscopic features of bronchiectasis

Answer 481

Inflammatory exudation within bronchi and bronchiole wall associated with desquamation of lining epithelium and extensive ulceration, bacteria found too

Question 482

List the macroscopic features of Group 2 PAH

Answer 482

Thickening of the capillary endothelial basement membrane Narrowing of the pulmonary arteries Smooth muscle hypertrophy (as a result of elastin production) Stiffening of the pulmonary vessels

Question 483

Define each T stage in the TMN staging system (general cancer)

Answer 483

Tis - carcinoma in situ T1 - Tumour ≤ 3cm T1a – ≤ 1cm T1b – 1-2cm T1c – 2-3cm T2 - Tumour 3-5cm T3 - Tumour 5-7cm T4 - Tumour > 7cm

Question 484

List diseases that can lead to Type 2 respiratory failure

Answer 484

``` COPD Severe asthma Drug overdose Poisoning Myasthenia gravis Polyneuropathy ``` Many more

Question 485

Describe the presentation of acute fibrotic lung diseases

Answer 485

Can present without PMH of lung disease or be part of an accelerated phase of underlying interstitial disease; most rapidly progress to respiratory failure.

Question 486

List the common complications of COPD

Answer 486

Respiratory infections - higher risk of catching them and also more vulnerable to further lung damage Heart problems - increases risk of heart disease, including heart attack Lung cancer - increased risk High blood pressure in lung arteries Depression

Question 487

How can O2 therapy be administered

Answer 487

``` Nasal cannula Face mask Non rebreather mask BiPAP/CPAP Intubation Tracheotomy ``` In acute cases it is given at the time, as required but in chronic cases they may have it long term

Question 488

Describe the features of simple coal worker's pneumoconiosis

Answer 488

Coal macules (1-2mm d.) consist of C-laden macrophages scattered throughout lung, upper lobes & upper zones of lower lobes in particular. Occurs next to respiratory bronchioles since this is site of initial dust accumulation

Question 489

What causes the cough seen in lung cancer

Answer 489

Central airway involvement

Question 490

Describe the prevalence of intrinsic lung diseases

Answer 490

Overall prevalence of 3-6 cases/100,000 people (US) Idiopathic pulmonary fibrosis (IPF) prevalence in UK is 50/100,000 Highest prevalence in adults >75 years old.  M>F

Question 491

The surgical treatments for cor pulmonale can have what complications

Answer 491

Venesection – nerve damage Pulmonary embolectomy – thrombosis and dissection Heart transplant – rejection, primary graft failure, cardiac allograft vasculopathy Lung transplant – rejection, bronchiolitis obliterans syndrome, post transplantation lymphoproliferative disorder

Question 492

What is Lambert-Eaton Syndrome

Answer 492

Muscle weakness due to autoimmune targeting of the neuromuscular junction

Question 493

How does cor pulmonale progress

Answer 493

It is a chronically progressive condition Patients have the features of the underlying lung disease before slowly progressing to CP Some cases such as those due to large PE can be acute and then acutely deteriorate into chronic CP

Question 494

What are the clinical features of bronchiectasis

Answer 494

Coughing up yellow or green mucus daily Shortness of breath that gets worse during flare-ups Fatigue Fevers and/or chills, Wheezing or whistling sound while you breathe Coughing up blood or mucus mixed with blood Chest pain from increased effort to breath Clubbing

Question 495

What are the 3 classic symptoms of pulmonary haemorrhage?

Answer 495

Dyspnoea, haemoptysis, iron deficiency anaemia

Question 496

List the characteristics of a malignant tumour

Answer 496

Harmful - includes cancer and often fatal Not localized Can invade and destroy adjacent structures and spread to distant sites (metastasize) Tend to adhere to any part that they seize on in an obstinate manner Typically have poorly defined margins and are less well-differentiated

Question 497

Describe the pathogenesis behind occupational lung diseases

Answer 497

Environmental trigger from occupational dusts etc. | However, only a small % of those exposed will develop the disease which implies there is also a genetic predisposition

Question 498

How can you manage hypersensitivity pneumonitis

Answer 498

Antigen avoidance | Recognise the antigen and reduce exposure to it

Question 499

What causes coal workers lung

Answer 499

Inhalation of coal particles and other admixed forms of dust | Carbon dust is main culprit

Question 500

How does silicosis appear on polarised microscopy

Answer 500

Nodules show weakly birefringent silicate particles

Question 501

What happens to the nucleus:cytoplasm ratio in malignancy

Answer 501

It approaches 1:1 instead of 1:4 to 1:6 | Nucleus gets larger

Question 502

Describe status asthmaticus

Answer 502

- Persistent asthma attack basically It is an exhausting process as huge respiratory effort is required (muscles tire fast) Unless you provide treatment (ventilation to reduce effort) it can be quickly fatal Quickly become hypoxic and can cause death (also high risk of arrhythmia)

Question 503

What is the function of O2 therapy in respiratory failure

Answer 503

To ensure adequate ventilation To reduce hypoxia To correct respiratory acidosis

Question 504

In patients with type 2 respiratory failure, what concentration of O2 should be given as treatment

Answer 504

Start at 24% and monitor CO2 levels

Question 505

How do you diagnose pulmonary haemorrhage

Answer 505

Bloods - FBC, coagulation studies, serology Radiography Bronchoscopy - assess bleeding site Lung biopsy - carried out if the less invasive tests cannot find a cause Further tests may be carried out if a specific cause is suspected

Question 506

Which other diseases does silicosis increase your risk of

Answer 506

TB - inhibits macrophages from killing phagocytised mycobacterium 2-fold increased risk of lung cancer.

Question 507

What is the FVC

Answer 507

The total amount of air exhaled forcefully and quickly after inhaling as much as you can

Question 508

Which lung cancers could be classed as N1

Answer 508

Those with metastasis to ipsilateral intraparenchymal, parabronchial or hilar lymph nodes

Question 509

Name a drug which can cause drug induced asthma

Answer 509

Aspirin

Question 510

The characteristics of usual interstitial pneumonia are seen in which diseases

Answer 510

IPF Connective tissue disorders Chronic hypersensitivity pneumonia Asbestosis Must distinguish from each other with clinical, laboratory and histological findings

Question 511

What does papillary lung adenocarcinoma look like

Answer 511

Finger like

Question 512

Describe the abnormal nuclear morphology seen in malignancy

Answer 512

Variable and often irregular nuclear shape Cancer cells: N:C ratio may approach 1:1 instead of 1:4 to 1:6 Chromatin is often seen Nuclei may be abnormally large

Question 513

What is the other name for Cryptogenic Organising Pneumonia (COP)

Answer 513

Bronchiolitis obliterans organising pneumonia (BOOP)

Question 514

Is there a genetic component to asthma

Answer 514

Yes - many polymorphisms involved | Generally involves mutations in genes for different mediators

Question 515

Can type 1 respiratory failure become type 2

Answer 515

Yes And vice versa Patients can also move in and out of respiratory failure

Question 516

Describe the pathogenesis of Group 4 PH (CTEPH)

Answer 516

Clots form where the initial PE was and adhere to the vessel walls (mostly in large or medium arteries). This causes stenosis and occlusion of the artery. Blood is diverted through other vessels and as a result their pressure increases. These vessels may then constrict and end up undergoing remodelling. Microvascular thrombi may also form. This all leads to a further increase in pressure and a progression of the disease (RHF).

Question 517

How do you treat lung adenocarcinoma

Answer 517

Surgery, radiotherapy, chemotherapy Responds well to immunological treatment if mutations in EGF-receptors are present

Question 518

How do you diagnose group 3 PH

Answer 518

Diagnostic tests are the same as all other groups Extra tests may be performed to confirm the underlying cause E.g. Pulmonary function for COPD or ILD and polysomnography for OSA

Question 519

How does coal worker's pneumoconiosis present

Answer 519

Usually benign | Little change to lung function even in severe cases

Question 520

What is a neoplasm

Answer 520

The collection of cells and stroma composing new growths

Question 521

What name is given to a benign neoplasm of the stratified squamous epithelium

Answer 521

Squamous cell papilloma

Question 522

What is the common outcome of all types of PH? (i.e. what does it progress to?)

Answer 522

Right-heart failure

Question 523

What may you find in the sputum sample of a patient with squamous cell lung cancer

Answer 523

Atypical cells may be found on sputum cytology

Question 524

How do you manage pulmonary artery seal and reperfusion pulmonary oedema which can be seen after CTEPH treatment

Answer 524

Supportive treatment – O2, ventilation and NO

Question 525

Signs and symptoms of cor pulmonale present early in the disease - true or false

Answer 525

False Symptoms of CP tend only to occur in advanced stages This is an issue if the underlying lung disease also has few symptoms - late diagnosis

Question 526

What can cause Cryptogenic Organising Pneumonia (COP)

Answer 526

It is a response to infection/inflammatory injury of lungs | Seen in transplant rejection

Question 527

How does sleep disordered breathing lead to Group 3 PH

Answer 527

Multifactorial - includes hypoxic vasoconstriction, hyperinflated lungs Related to the severity and duration of apnoea's as these episodes increase PA pressure

Question 528

How does PH affect survival in lung disease patients

Answer 528

Having PH alongside the underlying lung disease can reduce survival. For example, 5-year survival COPD patients without pulmonary hypertension is 62%, compared with 36% in those with both

Question 529

List the cytologic features of a benign neoplasia

Answer 529

``` Low nuclear to cytoplasmic ratio Contain regular cells Normochromatic nuclei Absent or scanty mitotic figures Only mild architectural disturbance with maintained polarity ```

Question 530

How can asbestosis cause pulmonary hypertension and cor pulmonale

Answer 530

Scarring can trap & narrow pulmonary arteries and arterioles Increases pressure

Question 531

List the potential medical treatments for diffuse parenchymal lung diseases (DPLDs)

Answer 531

Smoking cessation and good pulmonary hygiene Supplementary O2 for significant hypoxaemia Removal of exposure if indicated – e.g., occupational, HSP. Stop toxic medications Treat respiratory infections Pulmonary rehabilitation may improve exercise intolerance and QoL. Influenza and pneumococcal vaccinations encouraged.

Question 532

List the clinical features of CTEPH

Answer 532

Exercise intolerance Fatigue Progressive dyspnoea As pressure increases symptoms progress to: Chest discomfort Syncope Haemoptysis Oedema (and other symptoms of heart failure )

Question 533

Describe the course of chronic fibrotic lung diseases

Answer 533

Insidious and slowly progressive

Question 534

What can cause localised pulmonary haemorrhage

Answer 534

Secondary aspiration of blood following infection, tumours, bronchitis or bronchiectasis Infarction Infection (pneumonia, TB etc) Trauma (laceration or contusion) Pulmonary arteriovenous malformation or other congenital malforamtions Underlying weakness in the vessels Iatrogenic – e.g. following biopsy

Question 535

Which has the better prognosis usual interstitial pneumonia or non-specific interstitial pneumonia

Answer 535

Non-specific interstitial pneumonia

Question 536

What causes the chest pain seen in lung cancer

Answer 536

Extension into other thoracic structures

Question 537

List the diseases that cause severe airway obstruction that can be the underlying cause of respiratory failure

Answer 537

``` Epiglottitis Tumours of the trachea COPD Asthma CF ``` They can all cause hypoxia and hypercapnia

Question 538

Describe the structure of tumour giant cells

Answer 538

A single huge polymorphic nucleus/two or more large hyperchromatic nuclei

Question 539

Sarcoidosis is a diagnosis of exclusion - true or false

Answer 539

True | Huge variety of symptoms that can mimic other conditions

Question 540

What does acinar lung adenocarcinoma look like

Answer 540

Cuboidal or columnar epithelium

Question 541

What is bronchiectasis

Answer 541

A disorder where there is destruction of the smooth muscle and elastic tissue by inflammation stemming from persistent or severe infections This leads to permanent dilatation of bronchi and bronchioles

Question 542

How do you determine if the respiratory failure is acute or chronic

Answer 542

Determine the underlying cause and symptom presentation | Not ABGs

Question 543

List the main inflammatory cells involved in atopic asthma

Answer 543

Eosinophils Mast cells Neutrophils T lymphocytes

Question 544

List the common features of hypercapnia

Answer 544

``` Headache Peripheral vasoconstriction Tachycardia Bounding pulse Tremor/flap Papilloedema Drowsiness and confusion ```

Question 545

List the main mediators of atopic asthma

Answer 545

Th2 cytokines | IL-4, IL-5 and IL-13

Question 546

Respiratory failure is purely a chronic condition – true or false

Answer 546

False It can have a chronic onset and course but can also have an acute onset and short duration It all depends on the underlying cause

Question 547

Which diseases (general categories) can cause respiratory failure

Answer 547

Diseases that cause diffuse alveolar filling Severe airway obstruction Disorders of the peripheral nervous system, respiratory muscles and chest wall - causes inadequate ventilation Depression of the neural drive of respiration - drugs, structural changes or metabolic abnormalities

Question 548

List common complications of asthma

Answer 548

``` Growth delay and higher risk of learning difficulty in children Permanent narrowing of airways Medication side effects Higher risk of obesity Risk of A&E visits - asthma attacks Lingering cough Frequent sick days Higher risk of depression ```