Journal Article

Current knowledge regarding Asherson's syndrome

Pereira, Rosa MR, de Carvalho, Jozélio F, Shoenfeld, Yehuda

2009

Overview

Catastrophic antiphospholipid (Asherson's) syndrome (CAPS) is known to be a severe variant (1%) of antiphospholipid syndrome, with a high rate of mortality (50%). The distinguishing feature of CAPS is microvascular thromboses in the presence of antiphospholipid antibodies. Molecular mimicry between β2-glycoprotein I and infectious agents, endothelial cell activation and reduced fibrinolysis are the most frequently described pathophysiological mechanisms. Genetic risk factors have also been implicated in the onset of CAPS, although these have not yet been identified. There have been no randomized, controlled trials evaluating the efficacy of any medication on CAPS; however, when CAPS is suspected, aggressive multimodal treatment is required. Patients who receive a combination of anticoagulation therapy, glucocorticosteroids and plasma exchange with or without intravenous immunoglobulin show the best survival rates. Herein, we review the clinical and laboratory findings, diagnostic criteria, pathophysiology, treatment and prognosis of CAPS.

• Publisher: Taylor & Francis,Expert Reviews Ltd,Informa Healthcare
• Subject: antiphospholipid antibodies / Antiphospholipid syndrome / Asherson's syndrome / Care and treatment / catastrophic antiphospholipid syndrome / Cell activation / Clinical trials / Corticoids / Diagnosis / Endothelial cells / Fibrinolysis / Immunoglobulins / Intravenous administration / intravenous immunoglobulin / Microvasculature / Mortality / plasma exchange / Prognosis / Risk factors / Survival / thrombophilia