Abstract

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 Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added.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 age was 27 years (range 17-38 yr). Most patients had a previous unsuccessful obstetric history. In 7 of 14 (50%) cases with available past 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 HELLP syndrome in 8 (53%) patients, placental infarctions in 4 (27%), and pelvic vein thrombosis and myometrium thrombotic microangiopathy (TMA) in 1 (7%) case each. Mortality rate was high, not only for the mothers (46%), but also 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.