Sequential treatment of rituximab and belimumab in thrombotic thrombocytopenia purpura associated with systemic lupus erythematous: A respective case series and literature review

Abstract

Introduction: Thrombotic thrombocytopenia purpura (TTP) associated with systemic lupus erythematous (SLE), features the appearance of inhibitory autoantibodies against ADAMTS13. Rituximab and belimumab (BEL), as both targeting B cells, seem to be an optimal therapy to induce clinical remission, prevent relapse of disease and contribute to glucocorticoid induction. However, the clinical outcome of SLE-TTP treated with sequential therapy between rituximab and BEL remain elusive.

Case series: We reported the clinical outcomes of 4 patients diagnosed with SLE-TTP who were administrated a combination of corticosteroids, plasma exchange, and rituximab at stage of induction. BEL was utilized to rapidly reduce the reliance on these agents and prevent relapse of TTP at maintenance stage. Ultimately, 4 patients fully recovered with a SLE Disease Activity Index score of 0 and reached the goal at dose of prednisolone <7.5 mg/d without relapse.

Conclusion: Sequential treatment of rituximab and BEL could be an encouraging approach in treatment of SLE-TTP and rapid glucocorticoid reduction.

Keywords: belimumab; rituximab; sequential treatment; systemic lupus erythematous; thrombotic thrombocytopenia purpura.