title: 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.
authors.affiliation: a Department of Dermatology , University Hospital of Münster , Münster , Germany.
authors.forename: Paraskevi
authors.initials: P
authors.lastname: Gkogkolou
authors.affiliation: a Department of Dermatology , University Hospital of Münster , Münster , Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Ehrchen
authors.affiliation: a Department of Dermatology , University Hospital of Münster , Münster , Germany.
authors.forename: Tobias
authors.identifier: http://orcid.org/0000-0002-3631-8841
authors.initials: T
authors.lastname: Goerge
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D017152:Antibodies, Antiphospholipid / Q000627:therapeutic use*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D006801:Humans; D009336:Necrosis; D010956:Plasmapheresis; D000069283:Rituximab / Q000627:therapeutic use*; D012871:Skin Diseases / Q000150:complications / Q000175:diagnosis
chemical_list: D000974:Antibodies, Antinuclear; D017152:Antibodies, Antiphospholipid; D000069283:Rituximab
keywords: Lupus anticoagulans; anti-β2-glycoprotein; anticardiolipin; digital necrosis