Rare but deadly manifestation of systemic lupus erythematosus

Correspondence to Dr Sebastian Alejandro Mikulic; sebastian.mikulic@jax.ufl.edu Description A 26-year-old African–American woman with systemic lupus erythematosus (SLE) and end-stage renal disease due to lupus nephritis was sent to the hospital from the dialysis centre following oxygen saturation of 73% and fever of 101°F. The patient reported worsening dyspnoea and fatigue for 3 days, which led her to miss her routine dialysis session the day before hospitalisation. High doses of intravenous glucocorticoids is the cornerstone of therapy, along with additional immunosuppressive agents to sustain remission.3 Traditionally, cyclophosphamide has been the agent of choice.5 During the acute phase of DAH, immediate hemostasis can be achieved with bronchoscopic administration of intrapulmonary recombinant factor VIIa.6 Also in cases of refractory hypoxaemia, supportive care with extracorporeal membrane oxygenation has been described.7 Recent advances in our understanding of pathogenic mechanisms driving the development of DAH highlight the pivotal role played by B lymphocytes and humoral immunity.8 Thus, rituximab (anti-CD20 monoclonal antibody) has been applied successfully in multiple case reports9 and in our case as well. A rising red blood cell count in sequential bronchoalveolar lavage aliquots from the same location is considered diagnostic of DAH.