Severe antiphospholipid antibody syndrome - response to plasmapheresis and rituximab.

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Severe antiphospholipid antibody syndrome - response to plasmapheresis and rituximab.

J Dermatolog Treat. 2017 Jan 13;:1-8

Authors: Gkogkolou P, Ehrchen J, Goerge T

Abstract
Antiphospholipid antibody syndrome 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 rash, interdisciplinary treatment is needed. Here, we describe the case of a 39 year-old woman with a severe hard-to-treat antiphospholipid antibody syndrome 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.

PMID: 28084106 [PubMed - as supplied by publisher]

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