부산대학교 도서관

Systemic autoimmunity may develop in association with severe Wiskott-Aldrich syndrome (WAS). Here, we present a patient with WAS complicated by steroid-resistant systemic autoimmunity, which was successfully treated with rituximab. A Japanese boy suffered from recurrent infections and eczema, and was genetically diagnosed as having WAS at one month of age. When the patient was 11 months old, he started to develop systemic autoimmune symptoms including dermatitis, vasculitis and arthritis. Rituximab was markedly effective for these symptoms, whereas prednisolone and cyclosporine A were not. After remission of his autoimmunity, he underwent allogeneic umbilical cord blood transplantation as the curative therapy for WAS. As growing attention has been paid recently to humoral immunity in association with the autoimmunity of WAS, rituximab might be one of the key drugs for treating such autoimmunity.