Catastrophic Antiphospholipid Syndrome: Clinical and Laboratory Features of 50 Patients

作者：Ronald A. Asherson, Ricard Cervera, Jean‐Charles Piette, Josep Font, J. T. Lie, Arsinur Burcoglu, Ken Lim, Francisco José Munoz, Roger A. Levy, François Boué, Jérôme Rossert, Miguel Ingelmo · 发表于：Medicine · 年份：1998 · DOI：10.1097/00005792-199805000-00005 · 被引用次数：549 · 研究领域：Systemic Lupus Erythematosus Research、Peripheral Neuropathies and Disorders、Renal Diseases and Glomerulopathies

We analyzed the clinical and laboratory characteristics of 50 patients with catastrophic antiphospholipid syndrome (APS) (5 from our clinics and 45 from a MEDLINE computer-assisted review of the literature from 1992 through 1996). Thirty-three (66%) patients were female and 17 (34%) were male. Twenty-eight (56%) patients had primary APS, 15 (30%) had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 1 (2%) had rheumatoid arthritis. Mean age of patients in this series was 38 +/- 14 years (range, 11-74 yr). Three (6%) patients developed the clinical picture of catastrophic APS under the age of 15 years, and 11 (22%) were 50 years old or more. In 11 (22%) patients, precipitating factors contributed to the development of catastrophic APS (infections in 3, drugs in 3, minor surgical procedures in 3, anticoagulation withdrawal in 2, and hysterectomy in 1). The presentation of the acute multi-organ failure was usually complex, involving multiple organs simultaneously or in a very short period of time. The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Thrombocytopenia was reported in 34 (68%) patients, hemolytic anemia in 13 (26%), disseminated intravascular coagulation in 14 (28%), and schistocytes in 7 (14%). The following antibodies were de...