Abstract:Objective To investigate the clinical characteristics and treatment of systemic lupus erythematosus (SLE) complicated with diffuse alveolar hemorrhage (DAH). Methods A retrospective analysis was performed on the clinical data, laboratory examination, imaging features, treatment and outcome of 13 inpatients with SLE complicated with DAH in the Second Affiliated Hospital of Air Force Military Medical University from July 2015 to January 2021. Results Among 1 male patient and 12 female patients, the average age for admission was (52.4±13.0) years old, the average onset age of SLE was (32.0±10.0) years old, and the average duration of disease was (5.9±5.8) years. In all patients, mean SLE disease activity index (SLEDAI) was 10.3±1.2, and the prominent clinical manifestations were dyspnea (92.3%), fever (92.3%), hemoptysis (100.0%) and renal involvement(100.0%). All patients were treated with high-dose adrenal glucocorticoid, and some patients received plasma exchange and continuous intravenous hemodialysis. Five patients (38.5%) survived during 1 year follow-up, and 8 patients (61.5%) died and giving up treatment during hospitalization or within 1 week after discharge. There was a significant difference in therapeutic plasma exchange between survival group and death group (100.0% vs 12.5%, P<0.01). Conclusion SLE-DAH patients are often accompanied by renal damage, dyspnea, hemoptysis, vasculitis, low complement levels, low albumin levels, high SLEDAI score and high mortality. High-dose glucocorticoid pulse therapy and plasma exchange can get good results for the patients.