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.

CONCLUSION:

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: Guillaume Hanouna, Nathalie Morel, Du Le Thi Huong, Laurence Josselin, Danièle Vauthier-Brouzes, David Saadoun, Adrien Kettaneh, Kateri Levesque, Véronique Le Guern, François Goffinet, Bruno Carbonne, Zahir Amoura, Jean-Charles Piette, Jacky Nizard, Nathalie Costedoat-Chalumeau
• Journal: Rheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 52 Issue 9 Pg. 1635-41 (Sep 2013) ISSN: 1462-0332 [Electronic] England
• PMID: 23676524 (Publication Type: Journal Article)

Topics

• Abortion, Spontaneous (etiology)
• Adult
• Antiphospholipid Syndrome (complications)
• Catastrophic Illness
• Eclampsia (etiology)
• Female
• HELLP Syndrome (etiology)
• Humans
• Pregnancy