Author/Editor		Cervera, Ricard; Bucciarelli, Silvia; Praprotnik, Sonja; Šipek-Dolničar, Alenka
Title		Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the CAPS Registry
Type		članek
Source		J Autoimmun
Vol. and No.		Letnik 32, št. 3-4
Publication year		2009
Volume		str. 240-5
Language		eng
Abstract		Objecrive: To describe the clinical and laboratory features, as well as the precipitating factors, treatment and outcome of patients with catastrophic antiphospholipid syndrome (APS). Methods: We analyzed the 280 patients included until September 2008 in the website based international registry of patients with catastrophic APS (CAPS Registry) (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). Results: The entire series includes 201 (72%) female and 79 (28%) male patients with a mean age of 37 +/- 14 years (range, 11-60 years). A total of 129 (46%) patients suffered from primary APS, 112 (40%) from systemic lupus erythematosus, 14 (5%) from lupus-like disease, and 25 (9%) from other autoimmune diseases. The catastrophic episode was the first manifestation of the APS in 129 (46%) patients. A precipitating factor was reported in 53% of the patients. The first clinical manifestation at the time of the catastrophic episode was a pulmonary complication in 24% of the cases, a neurologic feature in 18% and a renal feature in 18%. During the catastrophic episode, intraabdominal involvement was identified in the majority of patients, mainly consisting of renal (71%), hepatic (33%), gastrointestinal (25%), splenic (19%), adrenal (13%), and pancreatic (8%) manifestations. 123 (44%) patients died at the time of the catastrophic APS event but the higher recovery rate was achieved by the combination of anticoagulants plus corticosteroids plus plasma exchange (PE) and/or intravenous immunoglobulins (IVIG) (69% versus 54%). Conclusion: The catastrophic APS is an uncommon but potentially life-threatening condition that needs high clinical awareness. The therapeutical connotation is that this may be corrected with the combination of anticoagulation plus steroids plus attempts at achieving a prompt reduction of antiphospholipid antibody titer (i.e. PE and/or IVIG).
Descriptors		ADULT ANTIBODIES, ANTIPHOSPHOLIPID ANTICOAGULANTS ANTIPHOSPHOLIPID SYNDROME CATASTROPHIC ILLNESS CHILD IMMUNOGLOBULINS, INTRAVENOUS PLASMA EXCHANGE REGISTRIES STEROIDS