Severe antiphospholipid antibody syndrome – response to plasmapheresis and rituximab

Abstract

Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disease characterized by arterial and/or venous thrombosis, recurrent abortions and detection of antiphospholipid antibodies. In fulminant cases, involvement of multiple organs can lead to significant morbidity and even fatal outcomes, so that a rapid, interdisciplinary treatment is needed. Here, we describe the case of a 39-year-old woman with a severe hard-to-treat APS with arterial occlusion and progressive skin necrosis, who was successfully treated with a combination therapy with plasmapheresis and rituximab. The treatment led to complete remission of the skin lesions for over a year. Clinical response correlated with a long-lasting reduction of antiphospholipid antibodies and B-cell depletion. This case demonstrates the use of antiphospholipid antibodies for monitoring APS-activity and shows that this severe vascular disease requires rigorous therapeutic approaches.

Keywords:

• Lupus anticoagulans
• anticardiolipin
• anti-β2-glycoprotein
• digital necrosis

Disclosure statement

The authors have no conflict of interest to declare.

Funding

This work was supported by Deutsche Forschungsgemeinschaft [GO1360/4-1].