Interstitial Lung Disease

Question 1

What occurs in idiopathic pulmonary fibrosis?
What are the signs?
What can be used to slow the disease down?

Answer 1

progressive fibrosis with no clear cause
insidious onset of SOB and dry cough over 3m

bibasal fine inspiratory crackles, clubbing, dry cough, SOB
life expectancy 2-5 years

pirfenidone (anti-fibrotic)
pulmonary rehabilitation

Question 2

What drugs can cause pulmonary fibrosis?

Answer 2

amiodarone
methotrexate
cyclophosphamide
nitrofurantoin

Question 3

What conditions can cause pulmonary fibrosis?

Answer 3

SLE
Rheumatoid Arthitis
systemic sclerosis
alpha-1-antitrypsin deficiency

Question 4

What happens in extrinsic allergic alveolitis?

Answer 4

A type III hypersensitivity reaction to an environmental allergen that causes parenchymal inflammation an destruction

Bronchoalveolar lavage: shows raised lymphocytes and mast cells

Question 5

What are examples of extrinsic allergic alveolitis?

Answer 5

Farmers lung
Bird fanciers lug
Mushroom workers lung
Malt lung

Question 6

What does asbestosis cause?

Answer 6

Lung fibrosis
Pleural thickening and pleural plaques
Adenocarcinoma
Mesothelioma

Question 7

How do you diagnose interstitial lung disease?

Answer 7

Spirometry (restrictive picture)
Reduced transfer factor
High resolution CT scan: ground glass appearance, bilateral interstitial shadowing
Can also confirm using biopsy and histology

ANA positive in 30%

Question 8

What are the causes of upper zone fibrosis?

Answer 8

TB SPACE
TuBerculosis
Sarcoidosis
Pneumoconiosis
Ank Spon
Cystic fibrosis
Extrinsic allergic alveolitis

Question 9

What are the causes of lower zone fibrosis?

Answer 9

ACID
Asbestosis 
Connective tissue disease
Idiopathic PF
Drugs