Diagnosis and management of catastrophic antiphospholipid syndrome.

Abstract:

INTRODUCTION:Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia. Expert commentary: CAPS is a rare variant of the antiphospholipid syndrome (APS). It is characterized by thrombosis in multiple organs and a cytokine storm developing over a short period, with histopathologic evidence of multiple microthromboses, and laboratory confirmation of high aPL titers. This review discusses the diagnostic challenges and current approaches to the treatment of CAPS.

journal_name

Expert Rev Hematol

authors

Carmi O,Berla M,Shoenfeld Y,Levy Y

doi

10.1080/17474086.2017.1300522

subject

Has Abstract

pub_date

2017-04-01 00:00:00

pages

365-374

issue

4

eissn

1747-4086

issn

1747-4094

journal_volume

10

pub_type

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