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Lupus
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Grand Rounds from International Lupus Centres `Catastrophic' antiphospholipid syndrome

P J Maddison

Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor LL57 2PW, UK. peter{at}madd10.freeserve.co.uk

C Thorpe

J R C Seale

W Ahmed

G S Whiteley

Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK

When `catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48year-old woman who presented with the `catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis.

Key Words: catastrophic antiphospholipid syndrome (CAPS) • antiphospholipid antibodies • acute respiratory distress syndrome (ARDS) • critical illness neuropathy

Lupus, Vol. 9, No. 7, 484-488 (2000)
DOI: 10.1177/096120330000900702


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F J Wiedermann, W Lederer, N Sepp, and W Schobersberger
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