title: Catastrophic antiphospholipid syndrome (CAPS).
abstract: Catastrophic antiphospholipid syndrome (CAPS) is a very severe variant of the classic APS, characterised by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of antiphospholipid antibodies (aPL), usually in high titre. Although patients with catastrophic APS represent less than 1% of all patients with APS, this is usually a life-threatening condition. In this article, we aimed to review the state-of-the art about current knowledge in pathogenesis, clinical manifestations, diagnosis and treatment strategies in CAPS.
authors.affiliation: Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, UK. savino.sciascia@unito.it
authors.forename: Savino
authors.initials: S
authors.lastname: Sciascia
authors.forename: Chary
authors.initials: C
authors.lastname: Lopez-Pedrera
authors.forename: Dario
authors.initials: D
authors.lastname: Roccatello
authors.forename: Maria Jose'
authors.initials: MJ
authors.lastname: Cuadrado
mesh_terms: D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000209:etiology / Q000276:immunology / Q000628:therapy; D002388:Catastrophic Illness; D006801:Humans; D011379:Prognosis; D035583:Rare Diseases / Q000175:diagnosis / Q000209:etiology / Q000276:immunology / Q000628:therapy