Catastrophic antiphospholipid syndrome (CAPS)

• Published in: Best practice & research. Clinical rheumatology Vol. 26; no. 4; pp. 535 - 541
• Main Authors: Sciascia, Savino, Lopez-Pedrera, Chary, Roccatello, Dario, Cuadrado, Maria Jose
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01.08.2012
• Subjects: Anticoagulation; Antiphospholipid syndrome; Antiphospholipid Syndrome - diagnosis; Antiphospholipid Syndrome - etiology; Antiphospholipid Syndrome - immunology; Antiphospholipid Syndrome - therapy; Catastrophic antiphospholipid syndrome; Catastrophic Illness; Humans; IVIG; Plasmapheresis; Prognosis; Rare Diseases - diagnosis; Rare Diseases - etiology; Rare Diseases - immunology; Rare Diseases - therapy; Rheumatology; IVIG; Anticoagulation; Catastrophic antiphospholipid syndrome; Antiphospholipid syndrome; Plasmapheresis
• ISSN: 1521-6942; 1532-1770
• DOI: 10.1016/j.berh.2012.07.005

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.