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Common pulmonary fibrosis exam questions for medical finals, OSCEs and MRCP PACES


Question 1. Causes of clubbing

What are the respiratory causes of clubbing?

  • Fibrosis (and TB)
  • Chronic suppurative lung disease (bronchiectasis; empyema; chronic abscess)
  • Lung cancel (not small cell: progresses too quickly)

Question 2. Causes of fibrosis

What are the causes of upper and lower zone fibrosis?

  • Upper lobe (‘A TEA SHOP’)
    • ABPA
    • TB
    • Exrinsic allergic alveolitis
    • Ankylosing spondylosis
    • Sarcoid
    • Histiocytosis
    • Occupational
      • Berylliosis, silicosis
    • Pneumoconiosis
  • Lower (‘BRAD’)
    • Bronchiectasis
    • RA (and other connective tissue disease)
    • Asbestos
    • Drugs

Question 3. Fibrosis exacerbations

What is an exacerbation of fibrosis?

  • An unexplained worsening or development of dyspnoea within 30 days with new radiological infiltrates on HRCT
  • Poor prognosis
  • Treatment is iv steroid as 1st line and other immunosuppressant (e.g. cyclophosphamide) as 2nd line

Question 4. Histiocytosis

What is histiocytosis?

  • A multi-system disease with abnormal proliferation of histiocytes. Commonly affects young people.
    • Respiratory
      • Mixed obstructive and restrictive pattern
      • Low KCO, low FEV1/FVC ratio
    • Renal damage
    • Skin
      • 1-5mm papules
    • Lymphadenopathy
    • Bones
      • Radiolucent skull lesions
      • Marrow invasion