Fibrosis

Question 1

pulmonary fibrosis

Clinical features

Prognosis

Management:
Med
Cons
Surg

Answer 1

Clinical features:

• insidious onset of shortness of breath
• dry cough over more than 3 months

Examination

• bibasal fine inspiratory crackles
• finger clubbing

Prognosis is poor with a life expectancy of 2-5 years from diagnosis.

Two medications are licensed that can slow the progression of the disease:

• Pirfenidone is an antifibrotic
• Nintedanib is a monoclonal antibody targeting tyrosine kinase and so is anti-inflammatory

Conservative

• Remove or treat the underlying cause
• Long term oxygen therapy
• Stop smoking
• Physiotherapy and pulmonary rehabilitation
• Pneumococcal and flu vaccine
• Advanced care planning and palliative care where appropriate

Surgical
Lung transplant is an option but the risks and benefits need careful consideration

Question 2

Drugs causing pulmonary fibrosis

Answer 2

Amiodarone
Cyclophosphamide
Methotrexate
Nitrofurantoin

Question 3

Causes of pulmonary fibrosis

Answer 3

Idiopathic 
Drugs
Asbestosis 
Hypersensitivity Pneumonitis (e.g. farmer's lung, bird fancier's lung)
Alpha-1 antitripsin deficiency
Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Systemic sclerosis

Question 4

What is hypersensitivty pneumonitis?

How is it diagnosed?

Managmeent

Examples of causes

Answer 4

Hypersensitivity pneumonitis is a type III hypersensitivity reaction to an environmental allergen that causes parenchymal inflammation and destruction in people that are sensitive to that allergen

Diagnosis:
- Bronchoalveolar lavage involves collecting cells from the airways during bronchoscopy by washing the airways with fluid then collecting that fluid for testing. This shows raised lymphocytes and mast cells in hypersensitivity pneumonitis.

Management

• removing the allergen
• giving oxygen where necessary
• steroids

Examples of specific causes:

• Bird-fanciers lung is a reaction to bird droppings
• Farmers lung is a reaction to mouldy spores in hay
• Mushroom workers’ lung is a reaction to specific mushroom antigens
• Malt workers lung is a reaction to mould on barley