Traitement par rituximab d’un purpura thrombopénique immunologique au cours d’un lupus systémique

Abstract

Immune thrombocytopenic purpura is an autoimmune disorder occasionally associated with systemic lupus erythematosus for which oral corticosteroids constitute the first-line treatment. Therapy may be complex, particularly in the event of a contraindication to the standard treatment, namely corticosteroids, splenectomy or immunosuppressants. We report the case of a patient with systemic lupus associated with immune thrombocytopenic purpura and multisystem tuberculosis. Because of a contraindication to corticosteroids, the patient was successfully treated with rituximab (anti-CD20 antibody). This medication (Mabthera) is indicated in the treatment of relapsing or refractory follicular lymphoma. A 31-year-old North African woman had been treated for 10 years with prednisone, hydroxychloroquine, methotrexate and non-steroidal anti-inflammatory drugs for systemic lupus erythematosus. She presented severe immune thrombocytopenic purpura (platelet count: 4G/l) 3 months after initiation of antitubercular treatment for multisystem tuberculosis. The patient was unsuccessfully treated at the outset with 3 infusions of intravenous immunoglobulin. Since thrombocytopenia remained under 5 G/l, she was given rituximab 375 mg/m2/week for 4 weeks. Thrombocytopenia and native anti-DNA antibody levels decreased after the third infusion (D16). No side effects of treatment were observed. The patient did not experience any relapse during the 29 months following the final infusion. In the present case, rituximab was used because of multisystem tuberculosis. Rituximab appears to constitute a safe and effective treatment for refractory immune thrombocytopenic purpura associated with SLE in patients having a contraindication to or refractory to conventional therapy.