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Catastrophic antiphospholipid syndrome

Nature Clinical Practice Rheumatology volume 2, pages 81–89 (2006)Cite this article

Abstract

Antiphospholipid syndrome (APS) is a thrombotic disorder associated with autoantibodies that target membrane phospholipids and phospholipid-binding proteins, which regulate coagulation. APS is usually characterized by major arterial or venous occlusions, pregnancy complications, or both. In 1992, Asherson described an unusual variant of APS termed the catastrophic antiphospholipid syndrome (also known as Asherson's syndrome), the hallmark of which is rapid multiorgan failure caused by widespread small-vessel thrombi. Empiric treatments have improved the prognosis of patients, but half still die from thrombotic diathesis, even though those who survive the acute stages frequently remain well. Given the persistently high mortality rate, efforts have been underway to facilitate early diagnosis, institute effective treatments in a timely manner and to better understand the cause (or causes) of this extreme condition in order to improve outcomes.

Key Points

  • The catastrophic antiphospholipid syndrome (CAPS; also known as Asherson's Syndrome) is a severe form of antiphospholipid syndrome

  • It is predominantly characterized by microangiopathy (small-vessel occlusions) but, in a third of patients, large arterial or venous thromboses might also occur; severe thrombocytopenia and hemolytic anemia are common

  • Because of significant clinical and pathologic overlap, it is proposed that a continuum exists between thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, the HELLP syndrome (hemolysis, elevated liver enzyme levels, low platelet count) and CAPS

  • A triggering event can be identified in 50% of reported cases of CAPS; these include infections, immunization, trauma, neoplasia or certain drugs

  • Early and aggressive multimodal treatment is critical, including parenteral anticoagulation, steroids and antibiotics (if indicated), plasma exchange, and intravenous immunoglobulin

  • Cytotoxic therapy with cyclophosphamide is often used, albeit without known benefits

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Figure 1: Similarities between microangiopathic syndromes and catastrophic antiphospholipid syndrome.
Figure 2: Thrombotic microangiopathy and antiphospholipid antibodies.

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Acknowledgements

The authors would like to acknowledge the members of the Catastrophic Antiphospholipid Syndrome (CAPS) Registry, including Ricard Cervera, Yehuda Shoenfeld, Jean-Charles Piette, Doruk Erkan, José Alfredo Gómez and Roger Levy, among others.

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Authors and Affiliations

  1. Oklahoma Medical Research Foundation Professor of Medicine at the University of Oklahoma, the Head of the Clinical Pharmacology Research Program of the Oklahoma Medical Research Foundation, and the Medical Director of the Lupus Foundation of America,

    Joan T Merrill

  2. Department of Medicine, Honorary Consultant Physician at the Rheumatic Disease Unit, University of Cape Town Health Sciences Centre in Cape Town, and a Consultant Rheumatologist at the Rosebank Clinic in Johannesburg, South Africa

    Ronald A Asherson

  3. He is also on staff as a regular Visiting Professor at the Systemic Autoimmune Diseases Unit, Hospital Clinic, in Barcelona, Spain

    Ronald A Asherson

Authors
  1. Joan T Merrill
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  2. Ronald A Asherson
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Correspondence to Joan T Merrill.

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Competing interests

Dr. Merrill is a consultant for Genentech, Biogen-IDEC who are developing rituximab for use in systemic lupus erythematosus, and is actively involved in ongoing trials of this agent.

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Merrill, J., Asherson, R. Catastrophic antiphospholipid syndrome. Nat Rev Rheumatol 2, 81–89 (2006). https://doi.org/10.1038/ncprheum0069

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