Ocular findings in a patient with hemophagocytic syndrome.

of high-dose steroids and an immunosuppressive agent. Therapy with intravenous cyclophosphamide is the agent best studied for SLE. Intravenous immunoglobulinhasalso proven tobebeneficial in selectedpatients.Theroleofmethotrexate in the treatment of SLE remains unproven, but itssafetyrecordmakes itanattractivealternative,especially for thecontrol of some steroid-refractory manifestations of SLE. Plasmapheresis could, by rapidly removing circulating immune complexes, provide acute relief in the severely ill patient in whom cyclophosphamide is not effective (case 1) or is undesirable (case 2). However, it could not have a lasting therapeutic effect unless it is combined with an immunosuppressive agent to retard the re-accumulation of immune complexes. Although randomized controlled clinical trials failed to document a generalized benefit of plasmapheresis when added to standard immunosuppressive therapy, patients with SLE who are in crisis seem to benefit from the concomitant use of plasmapheresis with systemic prednisone and sequential intravenous cyclophosphamide. To the best of our knowledge, this is the first report of patients with SLE retinal vasculitis who were successfully treated with a combination of plasmapheresis and immunosuppressive chemotherapy.