Current knowledge regarding Asherson's syndrome

Jozélio F. De Carvalho, Rosa M.R. Pereira, Yehuda Shoenfeld

Research output: Contribution to journalReview articlepeer-review

Abstract

Catastrophic antiphospholipid (Ashersons) 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 2-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.

Original languageEnglish
Pages (from-to)587-591
Number of pages5
JournalExpert Review of Clinical Immunology
Volume5
Issue number5
DOIs
StatePublished - Sep 2009

Keywords

  • Antiphospholipid syndrome
  • Ashersons syndrome
  • Catastrophic antiphospholipid syndrome
  • Intravenous immunoglobulin
  • Plasma exchange
  • Thrombophilia

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