"CAPS REGISTRY": A REVIEW OF 200 CASES FROM THE INTERNATIONAL REGISTRY OF PATIENTS WITH CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME (CAPS)

Background: Catastrophic antiphospholipid syndrome (CAPS) is an often life-threatening variant of the antiphospholipid syndrome (APS) characterized by multiple microvascular thrombotic events, presenting over a short period of time and causing multiorgan failure and, in many cases, death. Due to the rarity of its presentation -they represent less than 1% of all patients with APS-, an International Registry of patients with CAPS was created in 2000 supported by the "European Forum on Antiphospholipid Antibodies".Objectives: To describe and analyze the main characteristics of patients with CAPS, as they appear in the "CAPS Registry", including previous APS manifestations, precipitating factors, thrombotic manifestations, immunological and laboratory abnormalities, treatment and outcome.Methods: We undertook a review of the first 200 patients included in the website-based International Registry of patients with CAPS ("CAPS Registry") (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). Data from these patients were summarized using a standardized data form and all were analyzed using a SPSS 11.5 statististical program.Results: The mean age was 38 years (7-74 yr) with a predominant female gender (F 72%/M 28%). Fourty-four percent suffered from primary APS, 41% from systemic lupus erythematosus, 7% from lupus-like disease, and 2% from rheumatoid arthirits. Fifty-five percent of the patients had previous APS manifestations (deep vein thrombosis 19%, fetal losses 16%, thrombocytopenia 16%, livedo reticularis 9%, pulmonary embolism 9%, and cerebrovascular events 8%). In 54% of the cases a identifiable precipitating factor was detected, including infections (17%), surgery (10%), anticoagulation withdrawal (6%), neoplasms (5%), and obstetric complications (5%). The majority of patients manifested at time of CAPS with multiple organ involvement, including renal (72%), pulmonary (65%), cerebral (58%), cardiac (53%), skin (51%), hepatic (31%), and peripheral thrombosis (21%). Thrombocytopenia was found in 65% of the patients, hemolytic anemia in 32%, disseminated intravascular coagulation in 21% and schistocytes in 12%. The following antibodies were detected: aCL IgG (83%), aCL IgM (39%), lupus anticoagulant (75%), ANA (62%), dsDNA (32%) and anti-ENA (20%). Anticoagulation was used in 76% of the episodes, steroids in 73%, cyclophosphamide in 33%, plasma exchange in 28%, intravenous gammaglobulins in 15% and hemodialysis in 15%. Death ocurred in 48% of patients.Conclusion: The "CAPS Registry" is a website-based International Registry that allows the on-line review of updated information on this rare but often life-threatening condition.Citation: , volume , supplement , year 2003, page Session: Anti-phospholipid syndrome 2