Interstitial lung disease

Question 1

What is interstitial lung disease?

Answer 1

Umbrella term to describe conditions that affect the lung parenchyma causing inflammation and fibrosis

Question 2

Name 2 clinical features of ILD

Answer 2

• Dyspnoea on exertion
• Non-productive paroxysmal cough
• Abnormal breath sounds
• Restrictive pulmonary spirometry

Question 3

1) What appearance does a high resolution CT scan show in a patient with ILD?
2) When a high resolution CT is unable to diagnose, what can be done?

Answer 3

1) Reticulonodular shadowing and honeycomb lung/ground glass appearance
2) Lung biopsy for histological analysis

Question 4

Why is the prognosis poor and management is supportive in ILD (in most cases)?

Answer 4

The damage to the lungs is irreversible

Question 5

ILDs can broadly be characterised into 3 categories - what are these categories?

Answer 5

• Those with a known cause
• Those associated with systemic conditions
• Idiopathic

Question 6

1) Name an ILD with a known cause
2) Name an ILD that’s associated with a systemic condition
3) Name an ILD that’s idiopathic

Answer 6

1) Occupational (asbestosis, silicosis, cotton workers lung), drugs i.e. nitrofurantoin and amiodarone and methotrexate, hypersensitivity reactions i.e. hypersensitivity pneumonitis, infections i.e. TB, GORD
2) Sarcoidosis, rheumatoid arthritis, SLE, UC
3) Idiopathic pulmonary fibrosis, nonspecific interstitial pneumonitis

Question 7

Idiopathic pulmonary fibrosis
1) What is this condition?
2) How does it present?
3) What are the 2 main things seen on examination with this condition?
4) What 2 medication can slow down the disease progress?

Answer 7

1) Progressive pulmonary fibrosis with no clear cause
2) Insidious onset of shortness of breath and dry cough over more than 3 months, malaise, weight loss, arthralgia
3) Bibasal fine end-inspiratory crackles and finger clubbing
4) Pirfenidone and nintedanib

Question 8

1) What kind of drug is pirfenidone (2)?
2) Nintedanib is a monoclonal antibody against what?

Answer 8

1) Antifibrotic and anti-inflammatory
2) Tyrosine kinase

Question 8

Name a cause of secondary pulmonary fibrosis

Answer 8

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

Question 9

Hypersensitivity Pneumonitis/ Extrinsic Allergic Alveolitis (EEA)
1) What is EEA?
2) Give an example of a specific cause of EEA, and what is the causative allergen
3) How is it managed?

Answer 9

1) Type III hypersensitivity reaction to an environmental allergen that causes parenchymal inflammation and destruction
2)
- Bird-fancier’s lung - bird droppings
- Farmer’s lung - mouldy spores in hay
- Mushroom worker’s lung - specific mushroom antigens
- Malt worker’s lung - mould on barley
3) Avoidance of triggers

Question 10

Asbestosis
1) What is asbestosis?
2) Name a complication of asbestos inhalation

Answer 10

1) Lung fibrosis related to the inhalation of asbestos
2) Lung fibrosis, pleural thickening and pleural plaques, adenocarcinoma, mesothelioma