Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry"

J Autoimmun. 2009 May-Jun;32(3-4):240-5. doi: 10.1016/j.jaut.2009.02.008. Epub 2009 Mar 26.


Objective: 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") (

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%).

Conclusions: 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).

MeSH terms

  • Adolescent
  • Adult
  • Antibodies, Antiphospholipid / blood*
  • Anticoagulants / therapeutic use
  • Antiphospholipid Syndrome / blood
  • Antiphospholipid Syndrome / drug therapy*
  • Antiphospholipid Syndrome / immunology*
  • Antiphospholipid Syndrome / mortality
  • Catastrophic Illness
  • Child
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Male
  • Middle Aged
  • Plasma Exchange
  • Registries / statistics & numerical data*
  • Steroids / therapeutic use
  • Young Adult


  • Antibodies, Antiphospholipid
  • Anticoagulants
  • Immunoglobulins, Intravenous
  • Steroids