title: Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients.
abstract: OBJECTIVE
  To assess the main causes of death and the prognostic factors that influence mortality in patients with the catastrophic antiphospholipid syndrome (CAPS).
  
  
  METHODS
  We analyzed the case reports of 250 patients included in the CAPS Registry up to February 2005. To identify prognostic factors for CAPS, we compared the main clinical and immunologic features and the types of treatment in the patients who died with those features in the patients who survived.
  
  
  RESULTS
  Recovery occurred in 56% of the episodes of CAPS and death occurred in 44%. Cerebral involvement, consisting mainly of stroke, cerebral hemorrhage, and encephalopathy, was considered the main cause of death, being present in 27.2% of patients, followed by cardiac involvement (19.8%) and infection (19.8%). The only factor we identified that was prognostic of a higher mortality rate was the presence of systemic lupus erythematosus (SLE). A higher recovery rate was associated with combined treatment with anticoagulants (ACs) plus corticosteroids (CS) plus plasma exchange (PE) (77.8%), followed by ACs plus CS plus PE and/or intravenous immunoglobulins (69%). In contrast, concomitant treatment with cyclophosphamide did not demonstrate additional benefit.
  
  
  CONCLUSIONS
  Cerebral involvement (mainly consisting of stroke), cardiac involvement, and infections were considered the main causes of death in patients with CAPS. The presence of SLE was related to a higher mortality rate. According to the results of the present study, ACs plus CS plus PE should be the first line of therapy in patients with CAPS.
authors.affiliation: Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain.
authors.forename: Silvia
authors.initials: S
authors.lastname: Bucciarelli
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
authors.forename: Doruk
authors.initials: D
authors.lastname: Erkan
authors.forename: José A
authors.initials: JA
authors.lastname: Gómez-Puerta
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.forename: Josep
authors.initials: J
authors.lastname: Font
authors.forename: Ronald A
authors.initials: RA
authors.lastname: Asherson
mesh_terms: D000328:Adult; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000276:immunology / Q000401:mortality* / Q000628:therapy; D002388:Catastrophic Illness / Q000401:mortality; D002423:Cause of Death; D015897:Comorbidity; D005260:Female; D006801:Humans; D008297:Male; D009026:Mortality; D011379:Prognosis; D012042:Registries; D015996:Survival Rate; D016896:Treatment Outcome