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Abstract

Thirty one patients with antiphospholipid antibodies who developed multi-organ failure (“Catastrophic Antiphospholipid Syndrome”) are reviewed. Thirteen suffered from a ‘Primary’ antiphospholipid syndrome, 13 from defined SLE, 4 from ‘lupus-like’ disease and one from rheumatoid arthritis. In more than one third precipitating factors were evident (e.g. infections, major/minor surgical procedures, oral contraceptives). Death occurred in 60% of patients from a variety of causes (myocardial failure, ARDS, CNS causes or, often a combination). Disseminated Intravascular Coagulation was present in 8 of 31 patients. Plasmapheresis appeared to be useful in several who had not responded to conventional therapy (e.g. IV heparin, steroids, immunosuppression).

Keywords

antiphospholipid syndrome multi-organ failure disseminated intravasular coagulation

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References

Asherson

. The catastrophic antiphospholipid syndrome. J Rheumatol 1992; 19: 508–512

Wang

Pancreatitis related to antiphospholipid antibody Syndrome in a patient with systemic lupus erythematosus. J Rheumatol 1992; 19: 1123–1125

Perez

McClendon

Lie

. Primary antiphospholipid syndrome with multiorgan arterial and venous thromboses. J Rheumatol 1992; 19: 1289–1292

Cacoub

Malignant hypertension in antiphospholipid syndrome without overt lupus nephritis. Clin Exp Rheumatol 1993; 11: 479–485

Ghosh

Adult respiratory distress syndrome associated with antiphospholipid antibody syndrome. J Rheumatol 1993; 20: 1406–1408

Mazzucchelli

Catastrophic antiphospholipid syndrome (CAS): a rare manifestation of the antiphospholipid antibody syndrome. Clin Exp Rheumatol 1993; 11: 653–657

Cappell

. Esophageal necrosis and perforation associated with the anticardiolipin antibody syndrome. Am J Gastroenterol 1994; 89: 1241–1245

Cervera

Several acute multi-organ complications in the ‘primary’ antiphospholipid syndrome: it is appropriate to use the ‘catastrophic’ adjective. Clin Exp Rheumatol 1994; 12: 345

Egan

Munn

. Catastrophic antiphospholipid antibody syndrome presenting with multiple thromboses and sites of vascular necrosis. J Rheumatol 1994; 21: 2376–2379

10.

Hochfeld

Pregnancy complicated by primary antiphospholipid antibody syndrome. Obstet Gynecol 1994; 83: 804–805

11.

Jain

Chartash

Susin

Furie

. Systemic lupus erythematosus complicated by thrombotic microangiopathy. Sem Arthritis Rheum 1994; 24: 173–182

12.

Kupferminc

Lee

Green

Peaceman

. Severe postpartum pulmonary, cardiac and renal syndrome associated with antiphospholipid antibodies. Obstet Gynecol 1994; 83: 806–807

13.

Pettelot

Syndrome gravissime des Antiphospholipides. La Presse Med 1994; 23: 1401

14.

Ruffatti

A catastrophic antiphospholipid syndrome: the importance of high levels of warfarin anticoagulation. J Int Med 1994; 235: 81–83

15.

Lie

Kobayashi

Tokano

Hashimoto

. Systemic and cerebral vasculitis coexisting with disseminated coagulopathy in systemic lupus erythematosus associated with antiphospholipid syndrome. J Rheumatol 1995; 22: 2173–2176

16.

Arenas

Sindrome antifosfolipido primario devastador e insuficienda renal aguda. Nefrologia XV 1995; 375–378

17.

Kennedy

Catastrophic anti-phospholipid syndrome in the absence of Ig G anti-cardiolipin antibodies. 1996; (in Press)

18.

Parke

. The catastrophic antiphospholipid syndrome with multi-organ failure. 1996; (in Press)

19.

Neuwelt

Catastrophic antiphospholipid syndrome. Response to repeated plasmapheresis over three years. 1996; (in Press)

20.

Piette

. The catastrophic antiphospholipid syndrome: report of two cases. 1996; (in Press)

21.

Falcini

Catastrophic antiphospholipid syndrome in pediatric systemic lupus erythematosus. J Rheumatol 1996; (in Press)

22.

Belmont

Abramson

Lie

. Review: Pathology and pathogenesis of vascular injury in systemic lupus erythematosus: interactions of inflammatory cells and activated endothelium. Arthritis Rheum 1996; 39: 9–22

The Catastrophic Antiphospholipid Syndrome 1996: Acute Multi-Organ Failure Associated with Antiphospholipid Antibodies: A Review of 31 Patients

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