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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

Journal of Intensive Care Medicine, Vol. 21, No. 3, 144-159 (2006)
DOI: 10.1177/0885066606287041


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