title: Catastrophic antiphospholipid syndrome and pregnancy: an experience of 13 cases.
abstract: OBJECTIVE
  Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening disease caused by the onset of rapidly progressive and widespread small-vessel thromboses in the presence of aPLs. The aim of this study was to examine pregnancy-related CAPS.
  
  
  METHODS
  Retrospective series of 13 patients with pregnancy-related CAPS with special focus on the follow-up. RESULTS; Eleven patients had known APS and had been treated with low-molecular-weight heparin (n = 10), aspirin (n = 8), oral anticoagulants (n = 1), HCQ (n = 3) and/or steroids (n = 1) during pregnancy. The most frequent manifestations of CAPS were cutaneous (n = 11), hepatic (n = 11), renal (n = 10), cardiac (n = 8) and neurological (n = 5). CAPS usually followed haemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome (n = 12), which was associated with pre-eclampsia (n = 6) or with eclampsia (n = 3). No maternal death was observed. The perinatal mortality of 54% was related to prematurity with a mean gestational age of 26.6 weeks at onset of CAPS or HELLP syndrome. During a mean follow-up of 4.8 years (range 2-8 years), seven new pregnancies occurred in five patients and led to one miscarriage, four successful pregnancies and two HELLP syndrome with pre-eclampsia or eclampsia that occurred at 28 weeks gestation in both cases despite optimal treatment. No relapse of CAPS was observed. Two mothers suddenly died 2.5 and 6 years after CAPS.
  
  
  CONCLUSIONS
  The occurrence of HELLP syndrome in a patient with APS should raise the suspicion of CAPS in the following days, and anticoagulation should be maintained post-partum or post-abortum. Subsequent pregnancies are at very high risk.
authors.affiliation: Centre Hospitalier Universitaire Pitié-Salpêtrière, Paris Cedex 13, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Hanouna
authors.forename: Nathalie
authors.initials: N
authors.lastname: Morel
authors.forename: Du
authors.initials: D
authors.lastname: Le Thi Huong
authors.forename: Laurence
authors.initials: L
authors.lastname: Josselin
authors.forename: Danièle
authors.initials: D
authors.lastname: Vauthier-Brouzes
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Adrien
authors.initials: A
authors.lastname: Kettaneh
authors.forename: Kateri
authors.initials: K
authors.lastname: Levesque
authors.forename: Véronique
authors.initials: V
authors.lastname: Le Guern
authors.forename: François
authors.initials: F
authors.lastname: Goffinet
authors.forename: Bruno
authors.initials: B
authors.lastname: Carbonne
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Jacky
authors.initials: J
authors.lastname: Nizard
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
mesh_terms: D000022:Abortion, Spontaneous / Q000209:etiology*; D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications*; D002388:Catastrophic Illness; D004461:Eclampsia / Q000209:etiology*; D005260:Female; D017359:HELLP Syndrome / Q000209:etiology*; D006801:Humans; D011247:Pregnancy
keywords: antiphospholipid syndrome; catastrophic antiphospholipid syndrome; lupus; pregnancy; thrombosis