The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus

We address the relationship between reactive hemophagocytic syndrome (RHS), systemic lupus erythematosus (SLE) activity, and treatment in 4 female pat ients with SLE. Febrile pancytopenia was related to cytologically proven RH S in all patients. Followup was 45 +/- 7 months from RHS onset. No causal i nfection could be identified. Outcome could be classified as: (1) RHS onset during a SLE flare and complete efficacy of high dose steroids; (2) death despite therapy for concomitant severe RHS and active SLE; (3) severe RHS i n inactive SLE under immunosuppressants, with remission after steroid taper ing and cyclophosphamide withdrawal. Three patients were treated with intra venous IgG. We conclude that (I) when SLE is active, RHS should be consider ed a specific manifestation and treated with steroids; (2) RHS occurring in otherwise inactive SLE might be related to iatrogenic immunosuppression; ( 3) intravenous IgG treatment might be indicated in both situations.