Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

RESPIRATORY > RESTRICTIVE LUNG DISEASE > Flashcards

RESTRICTIVE LUNG DISEASE Flashcards

(88 cards)

Start Studying
1
Q

What drugs can cause ILD?

A

Amiodarone
Bleomycin and busulphan
Nitrofurantoin
Methotrexate
Cyclophosphamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is interstitial lung disease?

A

A term used to describe a number of conditions that primarily affect the lung parenchyma and are characterised by chronic inflammation and or progressive interstitial fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Whats the epidemiology of interstitial lung disease?

A

50 per 100,000 in the UK
IPF is most common with 6,000 new cases per year

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Whats the pathophysiology of interstitial lung disease?

A

Inflammation and fibrosis in the lung interstitium due to fibroblasts secreting excess extracellular matrix secondary to damage.
The lung interstitium becomes thicker, increasing the diffiusion distance and impairing gas exchange

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the 2 types of restrictive lung diseases?

A

Interstitial lung disease
Extra-pulmonary lung disease - stuctures around lungs are damage which prevents chest expansion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are examples of extrapulmonary causes of restrictive lung diseases?

A

Pectus excavatum or pectus carinatum
Obesity
Pleural effusion
Myasthenia gravis
Kyphoscoliosis
Diaphragmatic hernia
Ascites
Pleural thickening

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the classifications of interstitial lung diseases?

A

Those with known cause:
- occupational/envrironmental
- drugs
- hypersensitivity reactions
- infections
- GORD

Those associated with systemic disorders
- sarcoidosis
- rheumatoid arthritis
- SLE, systemic sclerosis, mixed connective tissue disease, Sjogren syndrome
- ulcerative colitis, renal tubular acidosis, autoimmune thyroid disease

Idiopathic :
- idiopathic pulmonary fibrosis
- cryptogenic organising pneumonia
- non-specific interstitial pneumonitis
- desquamative interstitial pneumonia
- acute interstitial pneumonia
- respiratory bronchiolitis-associated ILD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How can GORD cause interstitial lung disease?

A

Chronic micro aspiration can lead to inflammation and damage to the lung tissue
GORD also shares common risk factors with ILD such as smoking which may also contribute to the development of both conditions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are occupational/environmental causes of interstitial lung disease?

A

Silica dust
Asbestos
Coal dust
Beryllium
Radiation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What drugs can cause interstitial lung disease?

A

Nitrofurantoin
Amiodarone
Bleomycin
Sulfasalazine
Busulfan

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are examples of causes of hypersensitivity pneumonitis?

A

Bird-fanciers lung is a reaction to bird droppings
Farmers lung is a reaction to mouldy spores in hay
Mushroom workers’ lung is a reaction to specific mushroom antigens
Malt workers lung is a reaction to mould on barley
Air-conditioner or humidifier lung - exposure to mould and bacteria growing in AC and humidifying systems
Chemical-induced - paints, adhesives, foams (chemicals such as isocyanates)
Woodworkers lung - exposure to wood dust e.g. sawing wood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What infections can cause interstitial lung disease?

A

Viral - infleunza, adenovirus, CMV, HSV, COVID
Bacterial - mycoplasma pneumoniae, legionella pneumophila and TB
Fungal - aspergillosis, histoplasmosis, cocidiomycosis
Parasitic - schistosomiasis, toxoplasmosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How does sarcoidosis cause interstitial lung disease?

A

It triggers inflammation and the formation of granulomas in lung tissue which causes damage to the lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How does rheumatoid arthritis cause interstitial lung disease?

A

It affects up to 10% of people with RA
Believed to be an abnormal immune response which triggers inflammation and fibrosis in the lung tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How do connective tissue disorders cause interstitial lung disease?

A

ILD affects up to 30% of people with connective tissue disorders
Abnormal immune response triggers inflammation and fibrosis in the lung

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Whats the most common type of interstitial lung disease?

A

Idiopathic pulmonary fibrosis - 50% of all cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is hypersensitivity pneumonitis?

A

Aka extrinsic allergic alveolitis
An inappropriate immune response to an allergen = inflammatory response = fibrotic changes with an upper lobe predominance (type 3 hypersensitivity reaction)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Whats the pathophysiology of hypersensitivity pneumonitis?

A

3 stages:
1. Sensitisation phase - repeated exposure to a specific antigen leads to the development of an immune response with the production of IgG and activation of T cells
2. Acute inflammation phase - exposure to antigen triggers an acute immune response in the lungs characterised by the influx of immune cells into the alveolar space and interstitium
3. Chronic inflammation - if antigen exposure persists then non-caseating granulomas and fibrosis in lung tissue forms. This can cause irreversible lung damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are the clinical features of hypersensitivity pneumonitis?

A

Acute phase - 4-6 hours post exposure - fevers, rigors, myalgia, dry cough, dyspnoea, fine bibasal crackles
Chronic - finger clubbing, increasing dyspnoea, weight loss, decreased exercise tolerance, type 1 respiratory failure, cor pulmonale

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How do you diagnose hypersensitivity pneumonitis?

A

Bloods - FBC shows neutrophilia, raised ESR, ABGs, serum antibodies
CXR - upper zone fibrosis and honeycomb lung
CT chest - nodules, ground glass appearance, extensive fibrosis
Lung function tests - restrictive defect
Bronchoalveolar lavage - raised lymphocytes and raised mast cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

How does idiopathic pulmonary fibrosis typically present?

A

70 year old with exertional dyspnoea and dry cough and fatigue
Bilateral fine end-inspiratory crackles on auscultation, clubbing and acryocyanosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are characteristic changes on high-resolution CT seen in idiopathic pulmonary fibrosis?

A

Usual interstitial pneumonia :
Honeycombing
Reticular opacities
Traction bronchiectasis
Emphysema
Loss of lung volume
(Usually seen in bases and peripheries)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Whats the prognosis of idiopathic pulmonary fibrosis?

A

50% 5 year survival rate
2-5 year survival rate from diagnsosi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are some industrial dust diseases?

A

Coal workers pneumoconiosis
Silicosis
Asbestosis
Berylliosis
Byssinosis
Hypersensitive pneumonitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Which 2 medications can be used to slow the progression of idiopathic pulmonary fibrosis?
Pirfenidone - antifibrotic and anti-inflammatory Nintedanib - monoclonal antibody targeting tyrosine kinase
26
What is cryptogenic organising pneumonia?
Aka bronchiolitis obliterans organising pneumonia A type of interstitial lung disease that involves a focal area of inflammation of the lung tissue Can be idiopathic or triggered by infection, inflammatory disorders, medications, radiation, environmental toxins or allergens
27
Whats the most common cause of hypersensitivity pneumonia is?
Farmers lungs - main antigen is thermophilic actinomyces species or aspergillosis species from moldy hay Must be differentiates from ODTS in farmers
28
What are the differences between organic dust toxic syndrome and hypersensitivity pneumonitis?
Both resolut from exposure to organic dusts and share common symptoms of SOB and fever ODTS is caused by toxic effects of endotoxins in organic dusts and hypersensitivity pneumonitis is a type 3 hypersensitivity immune response to organic antigens ODTS is acute whilst HP can be acute or chronic ODTS is managed primarily supportively and HP may require corticosteroids
29
How does smoking affect the risk of hypersensitivity pneumonitis?
Decreases the risk - may be because nicotine is immunosuppressive
30
What are the differences in presentation between acute and chronic hypersensitivity pneumonitis?
Acute - fever, cough, dyspnoea and fatigue within 12 hours of exposure. Symptoms will resolve within 48 hours of removing exposure Chronic - progressive cough, dyspnoea, fatigue and weight loss. Characterised by fibrosis. Symptoms wont resolve fully with removal of exposure
31
Why are skin tests not helpful for hypersensitivity pneumonitis?
As skin tests test for IgE but in hypersensitivity pneumonitis it is actually an IgG mediated response
32
What are risk factors for idiopathic pulmonary fibrosis?
Cause isn’t known but thought to be some genetic risk factors involving surfactant, gel forming mucin and telomerase Being older Male sex Cigarette smoking and environmental exposures GORD - microaspirations Obstructive sleep apnoea Air pollution Herpes infection FHx
33
Whats the pathophysiology of IPF?
Recurrent microinjuries to alveolar epithelial and basement membrane Release of pro inflammatory cytokines and chemo kinase e.g. TNF alpha Activation of resident fibrocytes and recruitment of circulating fibrocytes Pro-fibrotic chemicals e.g. PDGF and TGF beta are secreted by alveolar cells which stimulates fibroblast activation and differentiation into myofibroblasts Myofibroblasts stimulate collagen synthesis Fibrotic foci form -> fibrosed lung
34
What are the features of idiopathic pulmonary fibrosis?
progressive exertional dyspnoea bibasal fine end-inspiratory crepitations on auscultation dry cough clubbing
35
What investigations should you do for IPF?
Bloods - CRP, ANA, rheumatoid factor ABG - hypoxia and hypercapnia likely Spirometry- restrictive pattern with reduced transfer factor CXR High resolution CT - investigation of choice and required for diagnosis
36
What might you see on x-ray for IPF?
CXR - small, irregular peripheral opacities - ground glass appearance that later progresses to honeycombing
37
How do you manage IPF?
Pulmonary rehabilitation Supportive care Pharmacological interventions - there is no conclusive evidence to support the use of any drugs to increase survival - Pirfenidone may be useful in selected patients Supplement oxygen lung transplant
38
Whats the prognosis for IPF?
3-4 years from diagnosis
39
How do you investigate for hypersensitivity pneumonitis?
imaging: upper/mid-zone fibrosis bronchoalveolar lavage: lymphocytosis serologic assays for specific IgG antibodies blood: NO eosinophilia
40
How do you manage hypersensitivity pneumonitis?
Avoid precipitating factors Oral glucocorticoids
41
When do you typically see radiation-induced pulmonary fibrosis?
Typically follows radiotherapy for breast or lung cancer and is usually seen 6-12 months after radiotherapy course
42
What are complications of idiopathic pulmonary fibrosis?
Type 2 respiratory failure Increased risk of lung cancer Cor pulmonale 50% mortality in 5 years
43
What is asbestosis?
Lower lobe interstitial lung fibrosis that manifests in patients 15-30 years following exposure to asbestos
44
How much exposure to asbestos do you need to get asbestosis?
A considerable amount - over a few months at least You need much less to cause development of mesothelioma although this is still very rare
45
How does asbestosis present?
Dyspnoea Cough Clubbing bilateral end-inspiratory crackles on auscultation Cyanosis Reduced chest expansion
46
How do you investigate asbestosis?
Pulmonary function tests - restrictive with reduced gas transfer pattern Chest X-ray High resolution CT Bronchoscopy and biopsy Open lung biopsy
47
What are chest X-ray findings caused by asbestosis?
Linear interstitial fibrosis Pleural plaques Pleural thickening Atelactasis
48
What are pleural plaques?
The most common form of asbestos-related lung disease Generally occur 20-40 years after exposure Benign and dont undergo malignant change
49
Which form of asbestos is the most dangerous?
Crocidolite (blue)
50
What is coal workers pneumoconiosis?
Aka black lung disease Occupational lung disease caused by long term exposure to coal dust particles 15-20 years after initialy exposure to coal dust
51
Whats the pathophysiology of pneumoconiosis?
Coal dust (2-5 μm in size) is inhaled and enters the lungs. The dust reaches the terminal bronchioles and there it is engulfed by alveolar and interstitial macrophages. The dust particles are then moved by the macrophages via the mucociliary elevator and removed from the body as mucus. In coal miners who are exposed over many years, the system is overwhelmed and the macrophages begin to accumulate in the alveoli, which starts an immune response, causing damage to the lung tissue.
52
What are the 2 ways which coal workers pneumoconiosis can present?
Simple pneumoconiosis Progressive massive fibrosis
53
What is simple pneumoconiosis?
The commonest type of pneumoconiosis Often asymptomatic Presence increases the risk of other lung disease May lead to progressive massive fibrosis
54
What is pneumoconiosis?
accumulation of dust in the lungs and the response of the bodily tissue to its presence
55
What is progressive massive fibrosis?
A severe and progressive form of pneumoconiosis Characterised by the development of large areas of fibrosis Round fibrotic masses are most commonly in the upper lobes. The exact pathogenesis is not known. Patients are often symptomatic and have both breathlessness on exertion and cough, some may have black sputum. Lung function testing shows a mixed obstructive/restrictive picture.
56
What is silicosis?
a fibrosing lung disease caused by the inhalation of fine particles of crystalline silicon dioxide (silica)
57
What is silicosis a risk factor for?
TB Lung cancer COPD Autoimmune disease Kidney disease
58
Why can silicosis cause secondary tuberculosis?
Silicosis can impair the function of macrophages so they are less effective at controlling TB infection
59
Which occupations are at risk of silicosis?
Mining Slate works Foundries Potteries
60
What are X-ray features of silicosis?
upper zone fibrosing lung disease 'egg-shell' calcification of the hilar lymph nodes
61
What is sarcoidosis?
A non-caseating granulomatous inflammatory condition that affects lungs, liver, eyes, skin, heart, kidneys, CNS, PNS, bones It’s of unknown aetiology
62
Who is sarcoidosis most common in?
Young adults 20-40 People of African descent Women (Other spike of incidence is in 60s)
63
Whats the most commonly affected organ by sarcoidosis?
Lungs (affects over 90% individuals)
64
How can sarcoidosis affect the lungs?
Mediastinal lymphadenopathy Pulmonary fibrosis Pulmonary nodules
65
How can sarcoidosis present?
Resp - cough, SOB, chest pain, wheeze Skin - erythema nodosum, lupus pernio Lymphadenopathy Fatigue, weight loss, malaise, swinging fever Joint pain and stiffness Eyes - uveitis, conjunctivitis
66
How can sarcoidosis affect the liver?
Liver nodules Cirrhosis Cholestasis
67
How can sarcoidosis affect the eyes?
Uveitis Conjunctivitis Optic neuritis
68
How can sarcoidosis affect the skin?
Erythema nodosum Lupus pernio Granulomas in scar tissue
69
How can sarcoidosis affect the heart?
Bundle branch block Heart block Myocardial muscle involvement
70
How can sarcoidosis affect the kidneys?
Kidney stones due to hypercalcaemia Nephrocalcinosis Interstitial nephritis
71
How can sarcoidosis affect the CNS?
Nodules Pituitary involvement e.g. diabetes insipidus Encephalopathy
72
What is Lofgren’s syndrome?
an acute form of sarcoidosis characterised by bilateral hilar lymphadenopathy, erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis
73
What is Mikulicz syndrome?
Form of sarcoidosis there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma Now outdated term and is included within the diagnosis of sjogrens syndrome
74
What is Heerfordt’s syndrome?
Aka uveoparotid fever Parotid enlargement, fever and uveitis Secondary to sarcoidosis
75
How do you investigate sarcoidosis?
Raised serum ACE Hypercalcaemia Raised serum soluble interleukin-2 receptor Raised CRP Raised immunoglobulins U&Es for kidney involvement Urine dipstick for urine ACR LFTs for liver involvement Ophthalmology for eye involvement ECG and echo for heart involvement Ultrasound abdo for liver and kidney involvement CXR - Hilar lymphadenopathy High resolution CT thorax MRI for CNS involvement PET scan for active inflammation Biopsy - histology showing non-caseating granulomas with epithelioid cells is gold standard
76
How do we manage sarcoidosis?
No treatment if no or mild symptoms as often resolves spontaneously Oral steroids 6-24 months (give bisphosphonates alongside) Second line - methotrexate or azathioprine Lung transplant is rarely requires in severe pulmonary disease
77
What are indications for steroid management of sarcoidosis?
patients with chest x-ray stage 2 or 3 disease who are symptomatic hypercalcaemia eye, heart or neuro involvement
78
Whats the prognosis of sarcoidosis?
Spontaneously resolves within 6 months in 60% of cases In a small number of pt it progresses with pulmonary fibrosis and pulmonary hypertension Death only rarely occurs when it affects the heart causing arrhythmias or the CNS
79
Why is diagnosis of cryptogenic organising pneumonia often delayed?
Due to its similarities to infective pneumonia - SOV, cough, fever, lethargy, CXR shows focal consolidation
80
How is a definitive diagnosis of cryptogenic organising pneumonia made?
Lung biopsy
81
How do you treat cryptogenic organising pneumonia?
Systemic corticosteroids
82
How is interstitial lung disease different from COPD and asthma?
These diseases affect the airways whereas interstitial lung disease affects the lung tissue itself Onset and progression of symptoms tends to be slower in interstitial lung disease
83
How does sarcoidosis cause hypercalcaemia?
Occurs in up to 20% of cases Granulomas can produce 1-alpha-hydroxylase which can convert 25-hydroxyvitamin D to its activate form = increased calcium absorption from the gut and increased bone resorption = hypercalcaemia Granulomas can also produce cytokines that stimulate osteoclasts
84
Why does sarcoidosis increase ACE?
The cells that make up granulomas are known to produce ACE Reason is not entirely known
85
What is TLCO?
Transfer factor for carbon monoxide (aka diffuse capacity of carbon monoxide) - a measure of the conductance of gas transfer from impaired gas to RBC Used to evaluate the function of the alveoli
86
What causes increased transfer factor?
Conditions where there is increased exposure of alveolar contents to blood e.g. pulmonary haemorrhage, polycythemia, left to right shunts
87
What is the transfer coefficient?
Transfer factor / alveolar volume
88
Why is transfer factor important?
if the patient has a disease that causes a decrease in lung surface area, or has had a lung removed, then there is a decrease in transfer factor but there is a normal KCO. However, in conditions such as fibrosing alveolitis or emphysema, where there is damage to the lung parenchyma there is a reduction in both transfer factor and transfer coefficient. On a similar note, if a reduction in lung volume is due to an inability to expand the thorax (e.g. weakness) then the TLCO is low but the KCO is normal or increased.

RESPIRATORY (22 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions