A 28-year-old woman with SLE develops progressive shortness of breath over 6 weeks. CT pulmonary angiography is negative for PE. HRCT shows bilateral lower lobe ground-glass opacities. BAL shows haemosiderin-laden macrophages. Complement levels are low. The MOST likely diagnosis is:
- A Lupus pneumonitis
- B Shrinking lung syndrome
- C Pulmonary hypertension
- D Diffuse alveolar haemorrhage (DAH) ✓
Explanation
Diffuse alveolar haemorrhage (DAH) in SLE presents with dyspnoea, haemoptysis (may be absent), bilateral infiltrates on imaging, and haemosiderin-laden macrophages on BAL — the hallmark finding. It is associated with active lupus (low complement, high anti-dsDNA). DAH carries a high mortality and requires urgent high-dose corticosteroids ± cyclophosphamide or rituximab. Shrinking lung syndrome presents with raised hemidiaphragms and restrictive pattern without alveolar infiltrates. Lupus pneumonitis does not produce haemosiderin-laden macrophages.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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