EXTENDED REPORT

¹ Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain
² Division of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg, South Africa
³ Unidad de Enfermedades Autoinmunes, HGR#36 IMSS Puebla, Departamento de Reumatología de la Facultad de Medicina, Benemérita Universidad Autónoma de Puebla, Puebla, México
⁴ Medical Clinic Department, Faculdade de Medicina, Universidade Federal de Mato Grosso do Sul, Campo Grande, Brazil
⁵ Rheumatology Division, University of São Paulo, São Paulo, Brazil
⁶ Department of Obstetrics and Gynecology, Moscow Medical Academy, Moscow, Russia

Correspondence to:
Dr R Cervera
Servei de Malalties Autoimmunes, Hospital Clínic, Villarroel 170, 08036-Barcelona, Catalonia, Spain; rcervera{at}clinic.ub.es

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 Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added.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

Abbreviations: 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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