This study is to describe our clinical experience of diffuse alveolar hemorrhage (DAH) in patients with systemic lupus erythematosus (SLE) in Taiwan. From July 1994 to June 2001, eight patients of DAH among 1541 different SLE patients (0.52%) admitted to the Chang Gung Memorial Hospital were included for chart review. Dyspnea (100%) and fever (87.5%) were the most common symptoms instead of hemoptysis (62.5%). The most common extrapulmonary presentation was renal involvement (100%), which included clinical nephritis, nephrotic syndrome or acute renal failure. The overall mortality rate was 50%. Two pregnant patients were successfully treated with combined plasmapheresis and continuous venovenous hemofiltration in addition to high dose corticosteroid. Analysis of the prognostic factors showed that the higher APACHE II (Acute physiology, Age and Chronic Health Evaluation) and organ system failure (OSF) scores, but not the SLE activity index (SLEDAI), were associated with the greater mortality. The higher serum creatinine level or the need of hemodialysis did not adversely affect the survival. In conclusion, DAH in SLE patients are often accompanied with multiple organ failure, aggressive immunosuppressive therapy and multiple modalities of extracorporeal organ support should be started early for a favorable outcome.