title: Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry.
abstract: OBJECTIVE
  To analyze the clinical and immunologic manifestations of patients with catastrophic antiphospholipid syndrome (CAPS) from the "CAPS Registry".
  
  
  METHODS
  The demographic, clinical and serological features of 500 patients included in the website-based "CAPS Registry" were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test while T-test for independent variables was used to compare groups regarding continuous variables.
  
  
  RESULTS
  500 patients (female: 343 [69%]; mean age 38±17) accounting for 522 episodes of CAPS were included in the analysis. Forty percent of patients had an associated autoimmune disease, mainly systemic lupus erythematosus (SLE) (75%). The majority of CAPS episodes were triggered by a precipitating factor (65%), mostly infections (49%). Clinically, CAPS was characterized by several organ involvement affecting kidneys (73%), lungs (60%), brain (56%), heart (50%), and skin (47%). Lupus anticoagulant, IgG anticardiolipin and IgG anti-β2-glycprotein antibodies were the most often implicated antiphospholipid antibodies (83%, 81% and 78% respectively). Mortality accounted for 37% of episodes of CAPS. Several clinical differences could be observed based on the age of presentation and its association to SLE. Those cases triggered by a malignancy tended to occur in older patients, while CAPS episodes in young patients were associated with an infectious trigger and peripheral vessels involvement. Additionally, CAPS associated with SLE were more likely to have severe cardiac and brain involvement leading to a higher mortality (48%).
  
  
  CONCLUSIONS
  Although the presentation of CAPS is characterized by multiorgan thrombosis and failure, clinical differences among patients exist based on age and underlying chronic diseases, e.g. malignancy and SLE.
authors.affiliation: Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
authors.forename: Ignasi
authors.initials: I
authors.lastname: Rodríguez-Pintó
authors.affiliation: Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
authors.forename: Marta
authors.initials: M
authors.lastname: Moitinho
authors.affiliation: Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
authors.forename: Irene
authors.initials: I
authors.lastname: Santacreu
authors.affiliation: Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Aviv, Israel.
authors.forename: Yehuda
authors.initials: Y
authors.lastname: Shoenfeld
authors.affiliation: Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, New York, NY, United States.
authors.forename: Doruk
authors.initials: D
authors.lastname: Erkan
authors.affiliation: Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.affiliation: Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain. Electronic address: rcervera@clinic.cat.
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
mesh_terms: D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000276:immunology*; D016736:Antiphospholipid Syndrome / Q000276:immunology*; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000276:immunology*; D012042:Registries
chemical_list: D017152:Antibodies, Antiphospholipid; D016682:Lupus Coagulation Inhibitor
keywords: Anticardiolipin; Antiphospholipid; Antiphospholipid antibodies; Antiphospholipid syndrome; Catastrophic antiphospholipid syndrome; Lupus anticoagulant