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


OBJECTIVE
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 Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added.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.
Gómez-Puerta
José A
JA
Servei de Malalties Autoimmunes, Hospital Clínic, Villarroel 170, 08036-Barcelona, Catalonia, Spain.
Cervera
Ricard
R
Espinosa
Gerard
G
Asherson
Ronald A
RA
García-Carrasco
Mario
M
da Costa
Izaias P
IP
Andrade
Danieli C O
DC
Borba
Eduardo F
EF
Makatsaria
Alexander
A
Bucciarelli
Silvia
S
Ramos-Casals
Manuel
M
Font
Josep
J
D000293:Adolescent; D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D002388:Catastrophic Illness; D005260:Female; D017359:HELLP Syndrome / Q000209:etiology; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis* / Q000628:therapy; D011256:Pregnancy Outcome; D011644:Puerperal Disorders / Q000175:diagnosis / Q000628:therapy; D012042:Registries