Hypersensitivity Pneumonitis

Acute or chronic hypersensitivity type III reaction to an antigen

Aetiology

  • Caused by an allergic reaction affecting the small airways and alveoli in response to an inhaled antigen or occasionally following ingestion of a causative drug
  • Common antigens include thermophilic bacteria (farmer's lung), avian proteins (bird fancier's lung), and fungi (malt worker's lung)
  • No cause identified in 30% of cases

Pathophysiology

  1. Inhaled antigens deposited in lung
  1. Stimulate antibody formation → immune complex formation (IgG) (type III hypersensitivity)
  1. Results in complement activation → inflammation (lymphocytic alveolitis)
  1. If chronic, persistent inflammation will lead to fibrosis

Clinical presentation

Acute

  • Symptom onset 4–6h following high intensity exposure
  • Malaise
  • Dry cough
  • Pyrexia
  • Dyspnoea
  • Examination often normal, may have crackles (no wheeze)

Chronic

  • Chronic HP results from repeated low dose antigen exposure over time
  • Usually no history of preceding acute symptoms
  • Insidious onset of respiratory and constitutional symptoms is typical - progressive dyspnoea, cough, and malaise
  • On examination - may be crackles, clubbing may be present (but unusual)

Investigations

Acute

  • CXR - widespread pulmonary infiltrates

Chronic

  • CXR - pulmonary fibrosis, most commonly in upper zones
  • CT - ground glass attenuation and patchy micronodules of fibrosis in the upper lobes
  • PFTs - restrictive (fibrosis)
  • Bloods - serum antibodies
  • Lung biopsy if in doubt

Management

Acute

  • Oxygen
  • Steroids
  • Antigen avoidance

Chronic

  • Antigen avoidance
  • Oral steroids if dyspnoea or low gas transfer
  • Antifibrotic therapy (pirfenidone, nintedanib)