Triple therapy with caplacizumab and rituximab for refractory systemic lupus erythematosus-associated thrombotic...

Systemic lupus erythematosus-associated thrombotic thrombocytopenic purpura is a rare but potentially life-threatening condition that is characterised by thrombocytopenia, microangiopathic haemolytic anaemia, and multiorgan involvement. Here, we describe a refractory case of systemic lupus erythematosus-associated thrombotic thrombocytopenic purpura in a 38-year-old Japanese woman who was unresponsive to plasma exchange and glucocorticoid therapy. Combination treatment with caplacizumab and rituximab leads to rapid platelet recovery, resolution of neurological symptoms, and sustained remission. Caplacizumab provided immediate inhibition of microthrombus formation, whereas rituximab targeted the underlying autoimmune process through B cell depletion. Following treatment, ADAMTS13 activity normalised, allowing for corticosteroid tapering and the maintenance of long-term remission. This case highlights the value of early combination therapy for refractory systemic lupus erythematosus-associated thrombotic thrombocytopenic purpura, and it suggests that early combination therapy with caplacizumab and rituximab may act synergistically to improve outcomes.