Efficacy and safety of rituximab in the treatment of primary antiphospholipid syndrome: Analysis of 24 cases from the bibliography review

Abstract

Background and objectiveAntiphospholipid syndrome (APS) is characterised by the presence of antiphospholipid antibodies (aPL) and thrombotic and/or obstetric manifestations. Patients without another associated autoimmune disease are considered to have primary APS. Some patients develop thrombosis recurrence despite anticoagulant treatment and some clinical features do not respond to standard therapy. Rituximab may be an alternative in these cases. We review the published scientific evidence on the use of rituximab in the treatment of primary APS. Patients and methodsDescription of a case and review of the literature with descriptive analysis of the demographic, clinical, and immunologic features, treatment and outcome of patients. ResultsWe identified 24 patients (15 women [62.5%]), with a mean age of 37.0±13.4 years. The reasons for the use of rituximab were thrombocytopenia (41.7%), skin involvement (33.3%), neurologic and heart valve involvement (12.5%), hemolytic anaemia (8.3%) and pulmonary and renal involvement (4.2%). Lupus anticoagulant was present in 72.7% of the cases, the IgG and IgM isotypes of anticardiolipin antibodies in 75 and 50%, respectively, and the anti-β2GPI (IgG and IgM) antibodies in 80% of the patients. Thirteen (54.1%) patients received two doses of 1000mg of rituximab fortnightly, 10 (41.7%) four doses of 375mg/m2 weekly and 1 (4.2%) eight doses of 375mg/m2 weekly. Eleven (45.8%) patients presented a complete clinical response, seven (29.2%) a partial response and six (25%) did not respond to rituximab. Four patients with clinical improvement presented with aPL titer decrease and in one patient, aPL levels did not change. In one patient without clinical response, aPL remained positive. A clinical-immunologic dissociation existed in two additional cases. ConclusionsThe results obtained suggest a possible potential benefit of rituximab in the treatment of some clinical manifestations of primary APS such as thrombocytopenia, skin and heart valve involvement.