Interstitial Lung Disease Flashcards

0
Q

What happens (in general) in interstitial lung disease?

A

Inflammation and fibrosis of the interstitium

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1
Q

What is the lung interstitium?

A

The potential space between the alveolar epithelium and capillary endothelium

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2
Q

What cells are involved in ILD?

A
Epithelial
Endothelial
Mesenchymal
Macrophages 
Recruited inflammatory cells
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3
Q

What type of deficit is ILD? Restrictive or obstructive? How?

A

Restrictive

-makes lungs less compliant due to development of the fibrous tissue

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4
Q

How is airway resistance affected in ILD?

A

It isn’t

Can sometimes be increased

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5
Q

What impairs gas exchange in ILD?

A

The lengthened diffusion pathway between alveolar air and blood impairs gas exchange

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6
Q

Clinical features of ILD?

A
4 D's
Dry cough
Progressive dyspnoea
Digital clubbing
Diffuse inspiratory crackles
Reduced exercise tolerance
Tachypnoea
Tachycardia
Bilateral reduced chest movement
Cyanosis and signs of right heart failure
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7
Q

Common general causes of ILD?

A
Occupational 
Treatment relayed 
Connective tissue disease
Immunological
Idiopathic
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8
Q

What are lung function results?

A

Show restrictive patterns.

FEV1/FVC is normal (>70%)

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9
Q

What is fibrosing alveolitis?

A

A progressive inflammatory condition of the lungs

Cause unknown

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10
Q

What happens histologically in fibrosing alveolitis?

A

Activated alveolar macrophages attract neutrophils and eosinophils
Lung damage due to ROS and proteases
Causes tissue destruction and fibrosis

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11
Q

Symptoms of fibrosing alveolitis?

A

Shortness of breath
Dry cough
Finger clubbing

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12
Q

What is seen on a CXR of fibrosing alveolitis?

A

Small lungs
Micro-nodular shadowing in lower lobes
Ragged heart borders

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13
Q

How is fibrosing alveolitis managed?

A

High dose oral steroids

Less effective once fibrosis has developed

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14
Q

What is extrinsic alveolitis?

A

A condition which results from repeated exposure to antigens of which the individual has been sensitised?

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15
Q

What are some examples of allergens in extrinsic alveolitis?

A

Mouldy hay (farmer’s lung)
Bird faeces (pigeon fancier’s lung)
Cotton fibres
Sugar cane fibres

16
Q

What happens in extrinsic allergic alveolitis?

A

Lymphocytes and macrophages infiltrate small airways after exposure
Can either resolve or lead to pulmonary fibrosis

17
Q

What happens in acute extrinsic alveolitis for eg farmer’s lung?

A

Exposed to allergen (thermophilic atinomyecytes)
Flu-like illness 4-9 hrs later with a dry cough and breathlessness on exertion
Fine mid and late inspiratory crackles
May be a wheeze

18
Q

What is seen in chronic extrinsic allergic alveolitis?

A

Insidious malaise
Dry cough and breathlessness over months/years
Inspiratory crackles

19
Q

What do lung function tests of extrinsic allergic alveolitis show?

A

Reduced compliance and reduced gas transfer

20
Q

What is asbestosis?

A

Interstitial lung disease caused by asbestos

21
Q

What is inhalation of asbestos associated with?

A

Benign pleural plaques
Mesothelioma
Asbestosis
Lung cancer

22
Q

Pathology of asbestosis?

A

Asbestos fibres are breathed in and penetrate alveoli to cause alveolitis.
Influx of macrophages which form characteristic asbestosis bodies

23
Q

Signs and symptoms of asbestosis?

A

Increased dyspnoea and exercise intolerance
Dry cough
Inspiratory crackles at lung bases which rise as the disease progresses
Lung function tests show small lungs, reduced compliance and impaired has transfer.

24
Q

Treatment of asbestosis?

A

There is none

25
Q

What is sarcoidosis?

A

A multi-system chronic inflammatory condition characterised by formation of epitheloid (macrophage) non-caseating granulomas at various sites in the body, commonly the lungs

26
Q

What cells do alveoli contain in sarcoidosis?

A

Lots of macrophages and lymphocytes

27
Q

What is seen on a CXR for sarcoidosis?

A

Miliary and modular shadowing

Diffuse fibrosis

28
Q

Treatment of sarcoidosis?

A

Steroids are usually effective in suppressing disease for stages 1-3

29
Q

What is seen in a lung function test for sarcoidosis?

A

Small lungs
Reduced compliance
Impaired gas transfer
May be airflow obstruction

30
Q

Name some occupation ska lung diseases and the environmental factors associated with it

A

Asthma - lab workers - rat urine

Diffuse fibrosis - boiler/pipe laggers, railway, construction - asbestos

Modular fibrosis - coal minor, demolition - coal dust, silica, asbestos

Alveolitis - farmer, pigeon fancier - fungal spores from hay, avian antigens

31
Q

What is seen in a CXR a for asbestosis?

A

Holly leaf plaques
Fibrosis
Mesothelioma

32
Q

What is seen on a CXR for acute and chronic extrinsic allergic alveolitis?

A

Acute - micro-nodule infiltrate, denser towards hila

Chronic - almost normal, progresses to fibrosis in later stages.

33
Q

What drugs can lead to ILD?

A

Methotrexate (rheumatoid arthritis)
Bleomycin (lymphoma treatment)
Amiodarone (arrhythmias)
Nitrofurantoin (antibiotic, UTIs)

34
Q

What are the immunological causes of ILD?

A

Sarcoidosis

Hypersensitivity pneumonitis

35
Q

What are some occupational causes of ILD?

A

Asbestosis
Silicosis
Coal workers pneumoconiosis