Hypersensitivity pneumonitis
Overview
Hypersensitivity pneumonitis (HP) / extrinsic allergic alveolitis - inflammatory lung disease caused by sensitised immune response to inhaled organic antigens. Upper and mid-zone distribution is the critical distinguishing feature from IPF (lower zone).
Pathophysiology
•Type III hypersensitivity (immune complex deposition) + type IV hypersensitivity (T-cell mediated granulomatous inflammation) - together cause alveolitis progressing to fibrosis if exposure continues
•Early allergen removal can fully reverse acute/subacute HP - unlike IPF where fibrosis is irreversible
Presentation
•Acute HP: fever, chills, myalgia 4-8 hours after antigen exposure; symptoms resolve within 24-72 hours of leaving the exposure environment
•Chronic HP: progressive exertional dyspnoea, dry cough, weight loss, bibasal inspiratory crackles, clubbing
Investigations
•Gold standard imaging: HRCT - upper and mid-zone ground-glass opacities, centrilobular nodules (acute/subacute); honeycombing and traction bronchiectasis (chronic fibrotic)
•BAL: lymphocytosis - characteristic and supportive of HP
•Serum precipitins (IgG): positive in up to 90% of farmer's lung and bird-fancier's lung; indicates sensitisation, not necessarily active disease
•Spirometry: restrictive pattern - reduced FVC, reduced TLCO, preserved/raised FEV1/FVC ratio
•Lung biopsy: non-caseating granulomas and lymphocytic alveolitis - if diagnosis uncertain
Management
🥇 First-line
•allergen identification and removal - most important intervention; full resolution possible in acute/subacute HP
🥈 Second-line
•oral prednisolone - if symptoms persist despite allergen avoidance, or in acute severe HP
🥉 Third-line
•azathioprine or cyclophosphamide - steroid-refractory or dependent disease
•Lung transplantation - end-stage fibrotic HP
Upper zone fibrosis - CHARTS mnemonic
•Lower zone fibrosis causes: IPF, asbestos, most connective tissue disorders (e.g. rheumatoid arthritis), drug-induced (methotrexate, amiodarone, bleomycin)
HP vs IPF - key comparison
HP vs IPF
| Feature | HP | IPF |
|---|---|---|
| Zone affected | Upper and mid-zone | Lower zone |
| Cause | Identifiable organic antigen | Idiopathic |
| BAL | Lymphocytosis | Normal / neutrophilia |
| Reversibility | Yes - if antigen removed early | No - irreversible |
| Serum precipitins | Positive (if sensitised) | Negative |