Prompt efficacy of plasmapheresis in a patient with systemic lupus erythematosus and diffuse alveolar haemorrhage

A 13-year-old girl, was admitted in the Paediatric Department of our Institute four months after the diagnosis of systemic lupus erythematosus (SLE), in poor general condition with fever, pallor, peripheral oedema, tachycardia, dyspnea and hemoptysis. Diffuse alveolar haemorrhage (DAH) in multiorgan SLE was diagnosed. The patient was initially treated with intravenous (i.v.) antibiotic, antiviral and antifungal therapy, pulse intravenous metilprednisolone i.v. (1 g/day for 5 days) and cyclophosphamide (1g), without significant benefit. She was then administered 3 courses of rituximab, with some clinical improvement and mild increase of Hb level . The girl was discharged on oral steroid therapy After one week, she was readmitted with poor general condition, severe dyspnea, pallor, low peripheral oxygen saturation (78%); Hb level was 4 g/dl. We decided to perform blood transfusions and plasmapheresis. After three plasmapheresis, we finally obtained persistant clinical remission. We suggest that plasmapheresis should be considered promptly as an effective treatment in this condition, in association with immunosuppressive treatment.