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Pulmonary fibrosis

A restrictive lung disease characterised by accumulation of excess fibrous connective tissue in the lung parenchyma, causing reduced lung compliance and oxygen diffusion capacity. This leads to progressive hypoxiaemia and shortness of breath.

Causes

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Clinical features

Symptoms

  • Chronic progressive shortness of breath
  • Dry cough

Signs of pulmonary fibrosis

  • Tachypnoea
  • Reduced expansion
  • Fine end-inspiratory crepitations 

Signs of cause

  • Clubbing (idiopathic pulmonary fibrosis)
  • Hand deformity (RA)
  • Telangiectasia, sclerodactyly and microstomia (systemic sclerosis)
  • Butterfly rash (SLE)
  • Lupus pernio (sarcoidosis)
  • Radiation burns
  • Kyphosis (ankylosing spondylitis)

Investigations

  • Chest X-ray: reticulonodular shadowing
  • High-resolution CT chest: honeycombing cysts, reticular septal thickening, ground glass changes (indicates inflammatory process which is often steroid responsive)
  • Spirometry: restrictive defect with reduced diffusion capacity (see interpretation notes on spirometry p251)
  • Blood tests: for likely causes, e.g. antinuclear antibody, rheumatoid factor, anti-centromere antibody
  • Lung biopsy

Management

NB: fibrosis is permanent and there is no cure.

  • Corticosteroids may help in acute exacerbations 
  • Antifibrotics (pirfenidone/nintedanib) may be used in idiopathic pulmonary fibrosis
  • Treat/withdraw cause if possible (may include corticosteroids and immunosuppressants)
  • Supplementary measures
    • Smoking cessation
    • Pulmonary rehabilitation  
    • Long-term oxygen therapy – usually considered if PaO2<7.3kPa (or <8kPa in presence of pulmonary hypertension or secondary polycythaemia)
  • Lung transplant (option for some patients)
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