Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases

• Published in: Annals of the rheumatic diseases Vol. 66; no. 6; pp. 740 - 746
• Main Authors: Gómez-Puerta, José A, Cervera, Ricard, Espinosa, Gerard, Asherson, Ronald A, García-Carrasco, Mario, da Costa, Izaias P, Andrade, Danieli C O, Borba, Eduardo F, Makatsaria, Alexander, Bucciarelli, Silvia, Ramos-Casals, Manuel, Font, Josep
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and European League Against Rheumatism 01.06.2007; BMJ; BMJ Publishing Group LTD; BMJ Group
• Subjects: aCL; Adolescent; Adult; anticardiolipin antibodies; Anticoagulants; antiphospholipid antibodies; antiphospholipid syndrome; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - diagnosis; Antiphospholipid Syndrome - therapy; aPL; APS; Autoimmune diseases; CAPS Registry; Catastrophic Antiphospholipid Syndrome Registry; Catastrophic Illness; central nervous system; Classification; CNS; DIC; disseminated intravascular coagulation; elevated liver enzymes; Extended Report; Female; haemolysis; HELLP; HELLP Syndrome - etiology; Hematology; Hospitals; Humans; Immunoglobulins; Internal medicine; Laboratories; low platelets; Lupus; lupus anticoagulant; Medicine; Miscarriage; Multiple Organ Failure - etiology; Obstetrics; Pregnancy; Pregnancy Complications - diagnosis; Pregnancy Complications - therapy; Pregnancy Outcome; Puerperal Disorders - diagnosis; Puerperal Disorders - therapy; Registries; Rheumatology; Thrombosis; thrombotic microangiopathy; thrombotic thrombocytopenic purpura; TMA; TTP
• DOI: 10.1136/ard.2006.061671
• ISSN: 0003-4967

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 Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added.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