Interstitial Lung Disease

Question 1

What is interstitial lung disease?

Answer 1

Conditions that affect the lung parenchyma (the lung tissue) causing inflammation and fibrosis

Question 2

Diagnosing interstitial lung disease

Answer 2

High resolution CT
Shows ‘ground glass’ appearance
Lung biopsy can be done if unsure

Question 3

Management of ILD (7)

Answer 3

Remove or treat the underlying cause
Home oxygen where they are hypoxic at rest
Stop smoking
Physiotherapy and pulmonary rehabilitation
Pneumococcal and flu vaccine
Advanced care planning and palliative care where appropriate
Lung transplant is an option but the risks and benefits need careful consideration

Question 4

What Idiopathic Pulmonary Fibrosis

Answer 4

Progressive pulmonary fibrosis with no clear cause
Presents with an insidious onset of shortness of breath and dry cough over more than 3 months
Usually 50+
Examination can show bibasal fine inspiratory crackles and finger clubbing
life expectancy of 2-5 years

Question 5

Medication that slows Idiopathic Pulmonary Fibrosis

Answer 5

Pirfenidone is an antifibrotic and anti-inflammatory

Nintedanib is a monoclonal antibody targeting tyrosine kinase

Question 6

What drugs cause Pulmonary Fibrosis?

Answer 6

Amiodarone
Cyclophosphamide
Methotrexate
Nitrofurantoin

Question 7

Secondary Pulmonary Fibrosis

Answer 7

Alpha-1 antitripsin deficiency
Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Systemic sclerosis

Question 8

Hypersensitivity Pneumonitis (AKA Extrinsic Allergic Alveolitis)

Answer 8

Type III hypersensitivity reaction
Parenchymal inflammation
Bronchoalveolar lavage shows raised lymphocytes and mast cells

Question 9

Effects of asbestos exposure

Answer 9

Lung fibrosis
Pleural thickening and pleural plaques
Adenocarcinoma
Mesothelioma