Systematic Review of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus Focus on Outcome and Therapy

Background Diffuse alveolar hemorrhage (DAH) is an uncommon but potentially life-threatening manifestation of systemic lupus erythematosus (SLE) associated with high mortality. Although survival and its associated clinical, laboratory, and therapeutic features have been reported for case reports and series, they have not been systematically reviewed. Objectives The purpose of this systematic review was to assess survival of episodes of DAH in SLE over 3 decades and to categorize trends in therapies, commonly utilized to treat this disorder. Results Overall, SLE patients survived 61% of 174 DAH episodes representing 140 patients. Episode survival was 67% in the time period from 2000 to 2013. Corticosteroids were nearly universally used therapeutically, and cyclophosphamide was used in 55%. Plasmapheresis was used in 31% and did not appear to be associated with survival. Conclusions Diffuse alveolar hemorrhage in SLE still carries a high risk of mortality; however, survival trends appear to demonstrate an increase from approximately 25% in the 1980s to 67% in the current decade. Increased use of cyclophosphamide appears to be associated with better survival, whereas plasmapheresis does not appear to influence outcome. Although these results need to be interpreted with caution because they are not derived from randomized controlled trials, we believe this represents the largest reported compilation of survival data in DAH associated with SLE.