title: Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases.
abstract: BACKGROUND
  The catastrophic variant of the antiphospholipid syndrome (APS) is a life-threatening form of presentation of this syndrome that can be triggered by several factors.
  
  
  OBJECTIVE
  To describe the characteristics of patients who developed catastrophic APS triggered during pregnancy and puerperium.
  
  
  METHODS
  A review of the first 255 cases collected in the website-based "CAPS Registry" was undertaken. Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added.
  
  
  RESULTS
  Fifteen cases were identified. The mean (range) age was 27 (17-38) years. Most patients had a previous unsuccessful obstetric history. In 7 of 14 (50%) cases with available medical history, the catastrophic APS appeared during pregnancy, in 6 (43%) during the puerperium and in 1 (7%) after curettage for a fetal death. The main clinical and serological characteristics were similar to those patients with catastrophic APS triggered by other factors, except for a history of a higher prevalence of previous abortions (p<0.01). Several specific features were found, including the HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome in 8 (53%) patients, placental infarctions in 4 (27%) patients, and pelvic vein thrombosis and myometrium thrombotic microangiopathy in 1 (7%) patient each. Mortality rate was high for the mothers (46%), and for the babies (54%).
  
  
  CONCLUSIONS
  It is important to consider the possibility of the development of catastrophic APS in those patients with signs of HELLP syndrome and multiorgan failure during pregnancy or puerperium, especially in those patients with previous history of abortions and/or thrombosis.
authors.affiliation: Servei de Malalties Autoimmunes, Hospital Clínic, Villarroel 170, 08036-Barcelona, Catalonia, Spain.
authors.forename: José A
authors.initials: JA
authors.lastname: Gómez-Puerta
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Ronald A
authors.initials: RA
authors.lastname: Asherson
authors.forename: Mario
authors.initials: M
authors.lastname: García-Carrasco
authors.forename: Izaias P
authors.initials: IP
authors.lastname: da Costa
authors.forename: Danieli C O
authors.initials: DC
authors.lastname: Andrade
authors.forename: Eduardo F
authors.initials: EF
authors.lastname: Borba
authors.forename: Alexander
authors.initials: A
authors.lastname: Makatsaria
authors.forename: Silvia
authors.initials: S
authors.lastname: Bucciarelli
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.forename: Josep
authors.initials: J
authors.lastname: Font
mesh_terms: D000293:Adolescent; D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D002388:Catastrophic Illness; D005260:Female; D017359:HELLP Syndrome / Q000209:etiology; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis* / Q000628:therapy; D011256:Pregnancy Outcome; D011644:Puerperal Disorders / Q000175:diagnosis / Q000628:therapy; D012042:Registries