Diffuse alveolar hemorrhage (DAH) is a rare life-threatening complication seen in patients with systemic lupus erythematosus (SLE). This paper describes the clinical features and outcome of seven SLE patients with DAH admitted to a medical intensive care unit (ICU) in a referral center. Of a total of 122 SLE patients, seven patients presented this complication (5.7%). Five patients were women (71.4%). Mean age was 24.3 (range 4-51 years). Mean duration of SLE before clinical DAH was 15.7 months (range 0-48 months). DAH was the initial manifestation of SLE in two patients (29%). DAH recurrence was seen in two patients (29%). Active lupus was present in all seven patients. Fever, glomerulonephritis, arthritis, myositis, and peripheral neuropathy were observed in six, four, four, three, and two patients, respectively. Five patients who underwent to bronchoscopy had positive findings of DAH (71.4%; i.e., bloody return on bronchoalveolar lavage--with hemosiderin-laden macrophages). Treatment options included intravenous methylprednisolone (10 mg kg(-1) day(-1)--3 days) following by prednisolone 1 mg kg(-1) day(-1) and pulse cyclophosphamide (750 mg/m(2)). Plasmapheresis was added in four patients (57.1%) because of the persistence of DAH. All patients were treated in an ICU, six of them requiring mechanical respiratory support (85.1%). Mortality rate during ICU stay was 12% (one patient). Our results show that DAH is an uncommon complication in SLE patients, requiring a prompt and aggressive recognition and treatment with high-dose steroid, intravenous cyclophosphamide, and plasmapheresis. With all of these treatments, there is a trend to a low mortality rate in patients with SLE presenting DAH.