AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'île de France, 27 Rue du Faubourg Saint Jacques, 75014, Paris, France.
Caen University Hospital, Internal Medicine Department, Caen, France.
Arthritis Res Ther. 2018 Nov 6;20(1):249. doi: 10.1186/s13075-018-1745-2.
Although one of the three obstetric manifestations of antiphospholipid syndrome (APS) is intrauterine fetal death (IUFD), little is known about it in this context. We report the first large series of patients with APS and IUFD.
We retrospectively analyzed the history and clinical data of women at four French hospitals. All had (1) APS diagnosis (Sydney criteria) and (2) IUFD at or after 10 weeks of gestation (weeks) between 2000 and 2016.
The study included 65 women. Their median age at the index IUFD was 29 years (IQR 26-33); 38 (58%) were primigravidas. The index IUFD was the first APS clinical manifestation in 48 women (74%). Overall, 35% had a triple-positive antibody profile. IUFD occurred at a median gestational age of 24 weeks (IQR 18-27) and was associated with maternal obstetric complications in 16 women (25%), namely, preeclampsia (n = 12), hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP) (n = 6), and/or placental abruption (n = 5). Half of the 50 women with available data had a small-for-gestational-age fetus. Overall, including during the follow-up period of 4 years (IQR 2-9), 28 women (43%) had at least one thrombosis, and 29% were diagnosed with systemic lupus erythematosus (SLE). Ultimately, 54 women (83%) had at least one live birth. Only one woman had three consecutive early miscarriages.
IUFD was most often the inaugural sign of APS. Of the APS classification criteria, IUFD, preeclampsia, and thromboses were common in this cohort, while the "3 consecutive early miscarriages" criterion was met only once. With treatment, most of the women successfully had at least one live birth.
尽管抗磷脂综合征(APS)的三种产科表现之一是宫内胎儿死亡(IUFD),但对此知之甚少。我们报告了首例 APS 合并 IUFD 的大型患者系列。
我们回顾性分析了 2000 年至 2016 年期间在法国四家医院就诊的女性的病史和临床数据。所有患者均(1)符合 APS 诊断(悉尼标准),(2)发生在 10 周妊娠后(周)的 IUFD。
研究共纳入 65 例女性。其首发 IUFD 的中位年龄为 29 岁(IQR 26-33);38 例(58%)为初产妇。48 例(74%)患者首发 IUFD 是 APS 的首次临床表现。总体而言,35%的患者抗体呈三阳性。IUFD 发生于中位妊娠 24 周(IQR 18-27),16 例(25%)孕妇合并产科并发症,包括子痫前期(n=12)、溶血性肝酶升高和血小板减少综合征(HELLP)(n=6)、胎盘早剥(n=5)。50 例有可用数据的患者中,有一半的胎儿小于胎龄。总体而言,包括 4 年的随访期间(IQR 2-9),28 例(43%)患者至少发生一次血栓,29%被诊断为系统性红斑狼疮(SLE)。最终,54 例(83%)患者至少有一次活产。仅有 1 例患者连续发生三次早期流产。
IUFD 是 APS 的首发表现。在本队列中,APS 的分类标准中,IUFD、子痫前期和血栓形成较为常见,而“连续发生三次早期流产”标准仅出现过一次。经治疗,大多数患者最终至少成功分娩一次。