Interstitial Lung Disease

Question 1

What is ILD?

Answer 1

Any disease process affecting lung interstitium (i.e. alveoli, terminal bronchi)

Question 2

What is idiopathic pulmonary fibrosis?

Answer 2

imbalance of fibrotic repair system

- more common in smokers

Question 3

What are the clinical signs of idiopathic pulmonary fibrosis?

Answer 3

progressive breathlessness (several years)
dry cough
clubbing
bilateral fine inspiratory crackles
CXR bilateral infiltrates
CT scan - reticulonodular fibrotic shadowing, worse at the lung bases, and periphery. Traction bronchiectasis. Honey-combing cystic changes

Question 4

What is the treatment of idiopathic pulmonary fibrosis?

Answer 4

No known medication - possibly anti fibronic drugs
Oxygen if hypoxic
Lung transplantation in young patients

Question 5

Describe Extrinsic Allergic Alveolitis

Answer 5

Type III hypersensitivity reaction to antigen lymphocytic alveolitis

Question 6

What are the causes of Extrinsic Allergic Alveolitis?

Answer 6

USUALLY OCCUPATIONAL
Thermophilic actinomycetes - farmers lung, malt workers, mushroom workers
Avian antigens - bird fanciers lung
Drugs - gold, bleomycin, sulphasalazine

Question 7

What are the symptoms of acute EAA?

Answer 7

cough, breathless, fever, myalgia
+/- pyrexia (temperature), crackles (no wheeze!), hypoxia
CxR: widespread pulmonary infiltrates

Question 8

What is the treatment of acute EAA?

Answer 8

oxygen
steroids
antigen avoidance

Question 9

What are the symptoms of chronic EAA?

Answer 9

breathlessness and cough
may be crackles
CXR: pulmonary fibrosis - most commonly in the upper zones
PFTs: restrictive defect (low FEV1 & FVC, high or normal ratio)

Question 10

What is the treatment of chronic EAA?

Answer 10

remove antigen exposure

oral steroids if breathless or low gas transfer

Question 11

What is Sarcoidosis?

Answer 11

Granulomatous (type 4 hypersensitivity) disease of unknown cause
- acute and chronic

Question 12

What is the treatment for Acute Sarcoidosis?

Answer 12

self-limiting condition - usually no treatment

Steroids if vital organ affected (eg impaired lung function, heart, eyes, brain, kidneys)

Question 13

What is the treatment for Chronic Sarcoidosis?

Answer 13

oral steroids usually needed

Immunosuppression (eg azathioprine, methotrexate, anti-TNF therapy)

Question 14

Name some Occupational Lung Diseases?

Answer 14

Coal Workers Pneumoconiosis
Silicosis
Asbestos Related lung disorders

Question 15

Describe the types of Coal Workers Pneumoconiosis?

Answer 15

Simple pneumoconiosis - chest X-ray abnormality only (no impairment of lung function)
Complicated pneumoconiosis - progressive massive fibrosis restrictive pattern with breathlessness.
Chronic bronchitis (coal dust + smoking)
Caplan’s syndrome - rheumatoid pneumoconiosis

Question 16

What does Chronic Bronchitis come from?

Answer 16

coal dust and smoking

Question 17

What causes Silicosis?

Answer 17

15-20 years exposure to quartz

eg mining, foundry workers, glass workers, boiler workers

Question 18

What are the characteristics of chronic Silicosis?

Answer 18

restrictive lung disease

pulmonary fibrosis

Question 19

What sort of jobs cause asbestos related lung disorders?

Answer 19

Mining, construction, shipbuilding, boilers and piping, automotive components

Question 20

Name the 4 progressive steps of Asbestos related lung disorders?

Answer 20

1- Benign pleural plaques - asymptomatic
2- Acute asbestos pleuritis - fever, pain, bloody pleural effusion
3- Pleural Effusion and Diffuse pleural thickening - restrictive impairment
4- Malignant Mesothelioma - incurable pleural cancer. Presents with chest pain and pleural effusion. No available treatment - fatal within two years.

Question 21

What diseases can Asbestos exposure lead to?

Answer 21

Pulmonary Fibrosis - “Asbestosis” - heavy prolonged exposure. Diffuse pulmonary fibrosis and restrictive defect. Asbestos bodies in sputum. Asbestos fibres in lung biopsy.

Bronchial carcinoma - asbestos multiplies risk in smokers

Question 22

What is the early stage of ILD?

Answer 22

alveolitis - injury with inflammatory cell infiltration

Question 23

What is the late stage of ILD characterised by?

Answer 23

fibrosis

Question 24

What are the causes of ILD?

Answer 24

Environmental (minerals, drugs, radiation. Post-ARDS)
-Hypersensitivity (mouldy hay, avian proteins)
Unknown (idiopathic)
-Connective tissue diseases
-Idiopathic pulmonary fibrosis (IPF)

Question 25

What biopsys may be done to diagnose ILD?

Answer 25

Transbronchial biopsy

Thoracoscopic biopsy

Question 26

What is the pathology of idiopathic pulmonary fibrosis?

Answer 26

Subpleural and basal fibrosis
Inflammatory component variable
Terminally lung structure replaced by dilated spaces surrounded by fibrous walls