The objective of this study was to determine the clinical features of systemic lupus erythematosus (SLE) patients with diffuse alveolar hemorrhage (DAH). The medical records of 29 patients with DAH among 2133 SLE patients admitted to Peking Union Medical College Hospital from January 2004 to January 2009 were reviewed in this retrospective study. It was found that hypoxemia (100%), dyspnea (90%), cough (79%) and fever (72%), rather than hemoptysis (66%), were the most common symptoms. The most common extrapulmonary presentation was renal involvement (90%). In about 60% of patients nosocomial infection was also present. The mean drop in hemoglobin was 32 ± 10.9 g/l. Mean Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was 17 ± 9. All patients received high-dose corticosteroid therapy and most were also given immunosuppressive agents. Rates of concomitant nosocomial infection and mechanical ventilation in non-survivors were higher than those of survivors (p < 0.05). DAH is a rare and catastrophic event in SLE. DAH can occur at any stage of the disease course; it usually occurs in patients with active SLE, and is frequently complicated by lupus nephritis. Some patients may have no hemoptysis. Early aggressive management with high-dose steroids and cyclophosphamide is advocated.