Respiratory Part 1 Flashcards
1
Q
What is sarcoidosis?
A
- This is a granulomatous disease of unknown cause
- It is multisystem but generally treated by respiratory team because 90% will have lung involvement
- Granulomas are non-caseating (contrast to TB)
2
Q
What are granulomas?
A
granulomas are nodules of inflammation full of macrophages
3
Q
Who tends to get sarcoidosis?
A
- Most common in young women in their 20-40s
- More common in those of Afro-Caribbean ethnicity
4
Q
Presentation of sarcoidosis?
A
- In the lungs it causes bilateral hilar lymphadenopathy, fibrosis and granulomas
- This presents as a restrictive defect – with symptoms of shortness of breath and a dry cough
- May also have general symptoms of fever, weight loss and fatigue
- Skin presentation includes erythema nodosum (inflammation of the subcutaneous fat) which presents as red, hard painful nodules on the shins
- In the eye it can cause optic neuritis, conjunctivitis and uveitis
- Sarcoidosis can also affect the heart, kidneys and CNS
- Neurosarcoidosis can cause a wide array of symptoms depending on which part of the nervous system it infiltrates, it can cause Bell’s palsy, Argyll Robertson pupil (a constricted pupil that accommodates but does not react to light)
5
Q
Classic skin lesions for sarcoidosis?
A
erythema nodosum - tender red nodules on the shins due to inflammation of subcutaneous fat
lupus pernio - erythematous indurated plaques
6
Q
Investigations for sarcoidosis?
A
- Diagnosis can sometimes be made on clinical grounds but generally requires confirmatory biopsy
- Initial bloods can be used to screen for the condition: serum ACE is raised, serum calcium may be raised, CRP will be raised and soluble IL-2 may be raised
- Imaging is likely to be done (as you would do in anyone presenting with a long history of cough and SOB), first a CXR which classically shows bilateral hilar lymphadenopathy, then may go on to do CT
- Would get a restrictive pattern on spirometry
- Bronchoscopy may be done for biopsy or alternatively a skin biopsy if skin lesions present
- “Non-caseating granulomas with epithelioid cells”
7
Q
Raised ACE?
A
sarcoidosis
8
Q
Management of sarcoidosis?
A
- Some patients with mild disease will not need any treatment
- Oral steroids are the first line treatment for sarcoidosis
- Next would be immunosuppressants such as methotrexate, azathioprine, leflunomide and mycophenolate
- Anti TNF therapy is third line e.g. infliximab, adalimumab
9
Q
Prognosis of sarcoidosis?
A
- In around 60% sarcoidosis resolves in 6 months
- However some may get pulmonary fibrosis and pulmonary hypertension and require a lung transplant
- If someone dies of sarcoidosis it is more commonly due to involvement of the heart causing arrhythmias
10
Q
Classic CXR appearance of sarcoidosis?
A
bilateral hilar lymphadenopathy
11
Q
Name 6 broad causes of interstitial lung disease?
A
- Dust diseases/ pneumoconiosis
- Hypersensitivity pneumonitis
- Secondary to a systemic condition
- Granulomatous – sarcoidosis/ TB
- Idiopathic – IPF/ COP
- Drug induced – amiodarone, methotrexate, cyclophosphamide, nitrofurantoin
12
Q
Explain what the dust diseases/ pneumoconioses are?
A
- Basically, a reaction to certain inhaled inorganic dusts including coal, silica and asbestos
- It is not an immune reaction like with the organic dusts in hypersensitivity pneumonitis, the inorganic dusts are actually toxic to the cells (so not everyone exposed to organic dusts will develop HP but basically everyone exposed to these inorganic dusts will develop lung disease)
13
Q
Is pure coal toxic? Explain what causes coal worker’s lung?
A
- Pure coal is actually inert and not toxic, damage in coal workers lung is due to inhalation of coal which has silicates and other pollutants in it (hence why coal is much less fibrinogenic than silica)
14
Q
Describe the 3 pulmonary syndromes that can result from inhalation of coal dusts?
A
- Simple Coal Worker’s Pneumoconiosis is simple deposition of coal in the airways with formations of some nodules which can be seen on XR, there is minimal functional loss so not many symptoms
- Progressive Massive Fibrosis patients develop round fibrotic masses particularly in the upper lobes and these masses sometimes have necrotic centres, this results in severe disability with dyspnea
- Caplan syndrome occurs in those with coal workers pneumoconiosis and rheumatoid arthritis
15
Q
Caplan syndrome =
A
coal workers lung plus rheumatoid arthritis
16
Q
Who may silicosis be found in?
A
- This is rare but can still be found in people working in stonemason (person who cuts stones for building), sand blasters, pottery and ceramics
17
Q
What is more fibrinogenic - coal or silica?
A
- Silica dust is highly fibrinogenic compared to coal dusts (i.e. less silica needs to be inhaled for lung disease to occur)
- Disease occurs even with small amounts of inhalation of silica
18
Q
In silicosis the ____ lobes are affected but in asbestosis the _____ lobes are affected
A
silicosis - upper lobes
asbestosis - lower lobes
19
Q
Distinctive XR appearance of silicosis?
A
eggshell calcification
20
Q
CXR shows eggshell calcification?
A
silicosis
21
Q
What type of asbestos fibres are most pathogenic?
A
chrysotile
22
Q
Jobs that may have or had asbestos exposure?
A
shipyard worker, construction worker, firefighters, industrial workers etc.
Asbestos was an excellent thermal and electrical insulator as well as being highly fire resistant so was used commonly in building materials
23
Q
Describe what happens in asbestosis?
A
- It causes diffuse fibrosis in the lower lobes often progressing to honeycombing
- severe SOB and persistent cough
- It has a very poor prognosis
24
Q
What workers may be exposed to berrylium and berrylliosis?
A
- This may occur in workers in electronics, high technology ceramics, dental alloy preparation and metals extraction
25
Management of dust diseases/ pneumoconiosis?
* There is no cure as damage already done
* Best management is prevention which is becoming better
* Need to stop any further exposure to the dust
* Stop smoking if are a smoker
26
Name 4 asbestos related lung conditions?
lung cancer
asbestosis
pleural plaques
mesothelioma
27
What 2 asbestos related condition require heavy exposure?
Lung cancer
Asbestosis
28
What 2 asbestos related conditions can occur even with light exposure?
Pleural plaques and mesothelioma
29
Describe asbestos and lung cancer risk?
* Associated with high asbestos exposure
* Those who have been exposed to asbestos have an increased risk of all histological variants of lung cancer especially if they smoke
* Risk of cancer = smoking x asbestos
30
Describe asbestos and pleural plaques?
* Can occur with only light asbestos exposure
* Mild pleural thickening and calcification causing some lung restriction but often asymptomatic
* Pleural plaques are the most common manifestation of asbestos exposure
* Those with pleural plaques can go on to develop other asbestos related issues too though
31
Describe asbestos and mesothelioma?
* Mesothelioma is malignant cancer of the pleura
* Mesothelioma will not develop till 20-40 yrs after the exposure
* Hence even though asbestos is no longer used in most things, there is still a relatively high number of cases
* Mesothelioma is pretty much always due to asbestos exposure
* Most common presentation is SOB, chest pain and weight loss, with finding of pleural effusion
* Mesothelioma has a very poor prognosis
32
Name 4 drugs that can cause pulmonary fibrosis?
amiodarone
methotrexate
nitrofurantoin
cyclophosphamide
33
Name 4 systemic conditions that may cause pulmonary fibrosis?
Alpha 1 antitrypsin deficiency, SLE, RA, systemic sclerosis
34
What is hypersensitivity pneumonitis?
* This is a type 3 hypersensitivity reaction that causes inflammation in the alveoli, the reaction is due to inhaled organic dusts
* Loads of different names depending on which organic dust e.g. bird fanciers lung (due to proteins on bird feathers), farmers lung (dust from mouldy hay), malt workers’ lung (mouldy barley)
35
Categorising hypersensitivity pneumonitis?
* Can present acutely, sub-acutely or chronically, however in practice these categories can be difficult to distinguish and it is more important to determine if there is fibrosis or not
36
Describe presentation of hypersensitivity pneumonitis?
* Acute presentation can mimic the flu with cough, breathlessness, fever and myalgia
* Subacute presentations may have background of many acute attacks
* Chronic presentations generally don’t have a background of acute attacks and present with progressive breathlessness and a dry cough
* On examination there are typically bibasal fine inspiratory crackles
37
Investigations for hypersensitivity pneumonitis?
* Bloods – inflammatory markers may be raised, serum IgG may be raised
* CXR
* CT
* Pulmonary function tests will show a restrictive pattern
* May consider bronchoalveolar lavage and biopsy
38
Management of hypersensitivity pneumonitis?
* Avoidance of trigger
* Oral steroids
* Should note that you cannot reverse established fibrosis
39
List some respiratory causes of clubbing (7)?
lung cancer
bronchiecstasis
lung abscess
empyema
IPF
mesothelioma
COP
40
What is idiopathic pulmonary fibrosis?
* Pulmonary fibrosis of unknown cause
* It is a chronic process that results ultimately in respiratory failure
41
Risk factors for IPF?
* The cause is unknown but being elderly, male and smoking increases risk
42
Presentation of IPF?
* Shortness of breath that gradually becomes worse
* A dry cough
* Finger clubbing
* Fatigue
43
Investigations for IPF?
* Need to exclude other causes of fibrosis e.g. occupational and environmental
* Spirometry
* CXR
* HR-CT – usually there is basal distribution of the disease with traction bronchiectasis (airways are pulled open) and honeycombing (small cystic air spaces and irregularly thickened walls)
* Histological confirmation may be necessary in some patients
44
Management of IPF?
* Supplemental O2
* Pulmonary rehab classes
* Anti fibrotic medication to slow down progress of disease – pirfenidone and nintedanib
* Lung transplant is only real cure but most are too old to have one
45
What are the 4 histological types of lung cancer?
small cell, squamous cell, adenocarcinoma, and large cell
46
Risk factors for lung cancer?
* Smoking is the biggest risk factor for developing lung cancer but other factors include occupational exposure to things such as asbestos, radiation, radon and uranium
47
How is lung cancer often categorised?
* Often lung cancer is categorised into small cell or non-small cell as small cell lung cancer is the most aggressive form and is more responsive to chemotherapy whereas the other forms are more commonly treated with surgery
48
# Small Cell Lung Cancer
* Arises from ______1______
* Usually arises ______2_____
* It is the most _______3_________
* It initially responds to _____4______
* The tumour often expresses ___5_____
* As it is a neuroendocrine tumour it can secrete _______6_____
1) immature (small) neuroendocrine cells
2) Centrally
3) aggressive form of lung cancer, and it metastasises early and widely
4) chemotherapy
5) NCAM-1
6) hormones such as ADH and ACTH
49
# Squamous Cell Carcinoma
* This is the most______1____ type of lung cancer
* These tend to arise ______2_________
* These tumours often produce ______3_______
* Squamous cell carcinoma can also cause ______4_______
1) common
2) centrally from major bronchi within dysplastic epithelium following squamous metaplasia (makes sense as this is where the smoke hits first)
3) keratin and express p40, p63 and cytokeratin
4) hypercalcaemia due to secretion of PTHrp
50
# Adenocarcinoma
* This is the most common tumour in _________1_____________
* Often adenocarcinomas arise __________2_____________
* The majority of these tumours express _____3_____
1) non-smokers (but adenocarcinoma is still common in smokers!) and is more common in women
2) in the periphery of the lung and sometimes in relation to scarring
3) TTF-1 or Napsin
51
Describe large cell carcinoma?
* No specific cell differentiation, this is a diagnosis of exclusion, the tumour usually arises centrally
52
Describe carcinoid tumours?
* These are low grade neuroendocrine tumours that if typical, generally do not metastasise
* Atypical carcinoid tumours however can metastasise
* They are not associated with smoking
53
List some local effects of lung cancer?
invasion of recurrent laryngeal nerve
invasion of pleura and chest wall
invasion of the pericardium
invasion of the brachial plexus
invasion of the sympathetic chain
invasion of the phrenic nerve
invasion of the superior vena cava
54
Describe superior vena cava obstruction in lung cancer presentation?
facial swelling, dyspnoea, distended veins in the neck and upper chest
Pembertons sign - raising hands over the head causes facial congestion and cyanosis
55
Describe presentation of invasion of the sympathetic chain in lung cancer?
this occurs with a pancoast (apical tumour)
patient presents with horner's syndrome
Ptosis, miosis (constricted pupil) and anhidrosis on one side of the face only
56
List some paraneoplastic effects of lung cancer?
finger clubbing
cushing syndrome
dilutional hyponatraemia
hypercalcaemia
hypertrophic pulmonary osteoarthropathy
thrombophlebitis
eaton lambert syndrome
57
Explain why lung cancer may present with cushing syndrome?
small cell lung cancer can secrete ACTH which results in adrenal hyperplasia and raised blood cortisol, symptoms and signs of cushing syndrome include central obesity but proximal muscle wasting, hypertension, increased blood glucose, striae (red stretch marks), round face, increased sweating, frontal balding in women
58
Explain why lung cancer may present with dilutional hyponatraemia?
small cell lung cancer can secrete ADH which results in increased water reabsorption therefore diluting the Na+ so concentration of Na+ decreases, this is called SIADH (syndrome of inappropriate ADH secretion)
59
Explain why lung cancer may present with hypercalcaemia?
squamous cell lung cancer can secrete PTHrp (parathyroid hormone related peptide) which mimics the real PTH released from the PTH. Normally PTH is released from the parathyroid when calcium levels are low so there can be more absorption of calcium from the gut and kidneys and calcium resorption from the bones, however if squamous cell lung cancer secretes PTHrp when the calcium is normal will get hypercalcaemia. Patient will have a high calcium but undetectable PTH.
60
Explain what Eaton lambert syndrome is?
This syndrome has many causes but it is mainly associated with lung cancer, it is an auto-immune condition where the body attacks the Ca gated sodium channels at the NMJ and get weakness in legs, arms and face (presents similarly to myasthenia gravis which also affects the NMJ)
61
Overview of management of lung cancer?
* SURGERY- this is the best option for cure but cannot be done if: the cancer has spread to the vocal cords, the cancer is closer than 2cm to the carina, the cancer is small cell, the cancer has metastasized. The patient also has to be fit enough to survive the surgery.
* CHEMOTHERAPY- this is used when the cancer has already spread, need to know type of cancer as small cell lung cancer responds a lot better to chemo
* RADIOTHERAPY- can be done palliatively to relieve symptoms or radically to cure the cancer
* PALLIATIVE CARE- much can be done to relieve distressing symptoms: a stent can be inserted to relieve stridor, radiotherapy done for painful bone metastases, pain killers
62
Explain what bronchiectasis is?
* Bronchiectasis is abnormal fixed dilation of the bronchi usually due to fibrous scarring following infection
* Due to the fixed dilatation the patient suffers from chronic lung infections
63
Causes of bronchiectasis?
* CF can cause bronchiectasis as mucus is stickier so clearance from the lungs in is impaired so pathogens aren’t swallowed and can cause disease
* Primary ciliary dyskinesia
* Secondary to an autoimmune disease
* Immunodeficiencies
64
Signs and symptoms of bronchiectasis?
* Persistent cough
* Sputum production (may be purulent)
* Recurrent infections
* Breathlessness
* Recurrent antibiotic prescriptions and short lived response
* Crackles on auscultation
* May have clubbing
65
Investigations for bronchiectasis?
* Sputum culture to identify colonising pathogens
* CXR to exclude other pathologies
* Spirometry to assess severity of airflow obstruction and identify any co-existent diagnoses
* Oxygen saturation levels
* FBC including WBC
* In secondary care high resolution CT will allow diagnosis: characteristically see tram track airways and signet ring sign
* Investigate for an underlying cause: CF testing, screen for antibody deficiencies, test for primary ciliary dyskinesia
* Depending on clinical features could also test for anti-CCP, ANA and ANCA, HIV serology, alpha-1 antitrypsin deficiency, GI investigations, serum protein electrophoresis, bronchoscopy
66
Management of bronchiectasis?
* No cure
* Smoking cessation
* Offer flu and pneumococcal vaccines
* Daily airway clearance therapies are advised (taught by physio)
* When people are colonized with persistent bacteria they may go on permanent low dose macrolides e.g. azithromycin
* Treat exacerbations as they occur
67
What is a pleural effusion?
* Excess accumulation of fluid in the pleural space
* Pleural Effusions can be transudative or exudatives
68
Describe a transudative pleural effusion?
* PROBLEM WITH PRESSURE – there is increased capillary hydrostatic pressure or decreased oncotic pressure that results in fluid being pushed across into the pleural space
* Causes include heart failure, nephrotic syndrome (plasma proteins lost in the urine), cirrhosis (decreased production of plasma proteins), constrictive pericarditis
* These have a low protein content as it is simply a passive process due to problems with pressure
* These tend to be bilateral as if you have a problem with pressure it’s generally not going to occur on only one side of your body
69
Describe an exudative pleural effusion?
* INFLAMMATORY RESPONSE, ACTUAL VESSELS ARE FAULTY AND LEAKY
* An exudative pleural effusion is cancer or infection until proven otherwise but can also occur in systemic inflammatory conditions such as SLE or in trauma
* This requires energy (e for energy and e for exudative) and these tend to be unilateral- you may only have infection in one lung or cancer in one lung
* As it is inflammatory there is a high protein content
70
Signs and symptoms of a pleural effusion?
* Breathlessness
* Cough
* Orthopnea (SOB when lying down- basically due to gravity when you are standing fluid collects at bottom but when lying it’s affecting the whole lung)
* Pleuritic chest pain
* Stony dull to percussion
* Reduced breath sounds
71
Investigations for a pleural effusion?
* Blurring of costophrenic angel on XR taken when patient standing up
* Aspiration done and tests carried out to determine if transudative or exudative
* Transudates: Protein < 30 g/l and LDH < 200 IU/L, fluid will be clear
* Exudates: Protein > 30 g/l and LDH > 200 IU/L, fluid will be cloudy
72
Lights criteria for exudative pleural effusion?
* Pleural fluid protein: serum protein > 0.5 (more than 2x)
* Pleural fluid LDH: serum LDH > 0.6
* Pleural fluid LDH > 2/3 upper limit of normal for serum
73
Management of a pleural effusion?
* Need to investigate and treat the underlying cause
* Treatment generally depends on cause and if they recur
* Can aspirate the effusions to dryness by talc
* Can insert a long term pleural catheter
74
Who should you suspect an empyema in?
* You should suspect this in someone with an improving pneumonia but ongoing fever
75
Management of empyema?
* These patients need a chest drain and antibiotics
76
CXR sign for empyema?
D sign
77
Lupus perinio?
rash on face with dark indurated plaques
happens in sarcoidosis
78
Kartageners syndrome causes
primary ciliary dyskinesia and also associated with dextrocardia
79
Explain the difference between CPAP and BiPAP and when they are used?
BiPAP helps someone to breathe but also sucks away excess CO2 - primary use is in type 2 mechanical respiratory failure (low O2 high CO2) usually related to COPD
CPAP is just continuous so it is more useful in type 1 respiratory failure e.g. acute heart failure as it pushes more oxygen in
80
Most common organism in an acute exacerbation of COPD?
haemophiliac influenza
81
Who gets antibiotics in acute exacerbation of COPD?
only those with purulent sputum or clinical signs of pneumonia
82
Moderate acute asthma criteria?
PEFR 50-75% best or predicted
Speech normal
RR < 25 / min
Pulse < 110 bpm
83
Severe acute asthma criteria?
PEFR 33 - 50% best or predicted
Can't complete sentences
RR > 25/min
Pulse > 110 bpm
84
Life threatening acute asthma criteria?
PEFR < 33% best or predicted
Oxygen sats < 92%
'Normal' pC02 (4.6-6.0 kPa)
Silent chest, cyanosis or feeble respiratory effort
Bradycardia, dysrhythmia or hypotension
Exhaustion, confusion or coma
85
Who do you give antibiotics to with acute bronchitis?
may consider if comorbidities etc
if CRP > 100 would prescribe
