Pefanis A, Williams D S, Skrzypek H, Fung A, Paizis K
Department of Nephrology, Austin Health, Melbourne, Australia.
Department of Obstetric, Medicine, Mercy Health, Melbourne, Australia.
Obstet Med. 2020 Mar;13(1):41-44. doi: 10.1177/1753495X18780853. Epub 2018 Jul 26.
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are rare small vessel vasculitides of unknown cause. The pathogenic role of MPO-ANCA in the vasculitides has been supported using various animal models, with B-cells playing a role in the disease pathogenesis. Pregnancy in the presence of an autoimmune disease such as vasculitis is often associated with significant morbidity. Little is known about the outcomes when women present with de novo vasculitis during pregnancy, and the appropriate management of such presentations is unclear. We describe a case of a 33-year-old female presenting in her second pregnancy with new onset ANCA vasculitis at 12 weeks' gestation. She was successfully treated with prednisolone and rituximab, and delivered a healthy 2.8 kg boy at 36 weeks' gestation with no clinical manifestations of vasculitis or neutropenia in the neonate.
抗中性粒细胞胞浆抗体(ANCA)相关血管炎是病因不明的罕见小血管血管炎。使用各种动物模型支持了MPO-ANCA在血管炎中的致病作用,其中B细胞在疾病发病机制中起作用。患有自身免疫性疾病(如血管炎)的孕妇通常伴有严重的发病率。对于孕期新发血管炎的女性患者的结局知之甚少,对此类情况的适当管理尚不清楚。我们描述了一例33岁女性,在第二次妊娠12周时出现新发ANCA血管炎。她接受泼尼松龙和利妥昔单抗治疗成功,并在妊娠36周时分娩了一个健康的2.8千克男婴,新生儿无血管炎或中性粒细胞减少的临床表现。