This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Vora, S. K. Articles by Erkan, D. Search for Related Content PubMed PubMed Citation Articles by Vora, S. K. Articles by Erkan, D. Social Bookmarking                   What's this?

Catastrophic Antiphospholipid Syndrome

Pulmonary Physicians of Norwich, Norwich, Connecticut

Ronald A. Asherson, MD

Division of Immunology, School of Pathology, University of Witwatersrand, Johannesburg, South Africa

Doruk Erkan, MD

Barbara Volcker Center for Women and Rheumatic Disease; Division of Rheumatology, Hospital for Special Surgery; and Weill Medical College of Cornell University, New York, New York, derkan{at}pol.net

Catastrophic antiphospholipid syndrome is a rapidly progressive life-threatening disease that causes multiple organ thromboses and dysfunction in the presence of antiphospholipid antibodies. A high index of clinical suspicion and careful investigation are required to make an early diagnosis so that treatment with anticoagulation and corticosteroids can be initiated; plasma exchange and/or intravenous immunoglobulins can be added if the life-threatening condition persists. Despite aggressive treatment and intensive care unit management, patients with catastrophic antiphospholipid syndrome have a 48% mortality rate, primarily attributable to cardiopulmonary failure. This article reviews the current information on the etiopathogenesis, clinical manifestations, diagnosis, management, and prognosis of catastrophic antiphospholipid syndrome.

Key Words: antiphospholipid syndrome • catastrophic antiphospholipid syndrome • anticardiolipin antibody • lupus anticoagulant • thrombotic microangiopathy • critical care • intensive care unit • multiple organ dysfunction syndrome

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				S van Wissen, B. Bastiaansen, A. Stroobants, E. van den Dool, M. Idu, M Levi, and E. Stroes Catastrophic antiphospholipid syndrome mimicking a malignant pancreatic tumour - a case report Lupus, June 1, 2008; 17(6): 586 - 590. [Abstract] [PDF]