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Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases
  1. José A Gómez-Puerta1,
  2. Ricard Cervera1,
  3. Gerard Espinosa1,
  4. Ronald A Asherson2,
  5. Mario García-Carrasco3,
  6. Izaias P da Costa4,
  7. Danieli C O Andrade5,
  8. Eduardo F Borba5,
  9. Alexander Makatsaria6,
  10. Silvia Bucciarelli1,
  11. Manuel Ramos-Casals1,
  12. Josep Font1,
  13. for the Catastrophic Antiphospholipid Syndrome Registry Project Group/European Forum on Antiphospholipid Antibodies*
  1. 1Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain
  2. 2Division of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg, South Africa
  3. 3Unidad de Enfermedades Autoinmunes, HGR#36 IMSS Puebla, Departamento de Reumatología de la Facultad de Medicina, Benemérita Universidad Autónoma de Puebla, Puebla, México
  4. 4Medical Clinic Department, Faculdade de Medicina, Universidade Federal de Mato Grosso do Sul, Campo Grande, Brazil
  5. 5Rheumatology Division, University of São Paulo, São Paulo, Brazil
  6. 6Department of Obstetrics and Gynecology, Moscow Medical Academy, Moscow, Russia
  1. Correspondence to:
    Dr R Cervera
    Servei de Malalties Autoimmunes, Hospital Clínic, Villarroel 170, 08036-Barcelona, Catalonia, Spain; rcervera{at}


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.

Aim: 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 Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added.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.

  • aCL, anticardiolipin antibodies
  • aPL, antiphospholipid antibodies
  • APS, antiphospholipid syndrome
  • CAPS Registry, Catastrophic Antiphospholipid Syndrome Registry
  • CNS, central nervous system
  • DIC, disseminated intravascular coagulation
  • HELLP, haemolysis, elevated liver enzymes, low platelets
  • LA, lupus anticoagulant
  • TMA, thrombotic microangiopathy
  • TTP, thrombotic thrombocytopenic purpura

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