Lee Rachel, Gonsalves Zenobia, Wang Sophia, Hussain Ayesha, Herrera Kimberly
Department of Obstetrics, Gynecology and Reproductive Medicine, Division of Maternal Fetal Medicine, Stony Brook University Hospital, Stony Brook, NY, USA.
Department of Obstetrics and Gynecology, Stony Brook University Hospital, Stony Brook, NY, USA.
Case Rep Perinat Med. 2024 Apr 26;13(1):20240007. doi: 10.1515/crpm-2024-0007. eCollection 2024 Jan.
Autoimmune hepatitis (AIH) is a chronic inflammatory disease of unknown etiology and AIH in pregnancy is associated with many adverse maternal and fetal outcomes. The purpose of this report is to share insight into management of AIH-induced pancytopenia unresponsive to steroids and transfusions.
A 29-year-old G4P0121 female with history of spontaneous bacterial peritonitis (SBP) and severe pancytopenia secondary to AIH was found to be incidentally pregnant at 7 weeks gestation. Despite multiple blood transfusions and steroids, her pancytopenia was unresponsive to therapy. At 33 weeks, she underwent primary cesarean section for persistent category II fetal heart tracing and delivered a viable infant. Delivery was complicated by hemorrhage requiring multiple blood products. Postpartum course was complicated by sepsis secondary to urinary tract infection, and decompensated cirrhosis with hepatic encephalopathy and coagulopathy. Both fetus and mother have recovered well 3 months post-delivery.
This case highlights the challenges in management of AIH in pregnancy, particularly the difficulty in treating severe unresponsive pancytopenia as well as balancing the need for immunosuppression with the increased risk of infection that may lead to sequelae such as SBP and puerperal sepsis.
自身免疫性肝炎（AIH）是一种病因不明的慢性炎症性疾病，妊娠合并AIH与许多不良母婴结局相关。本报告的目的是分享对AIH所致对类固醇和输血无反应的全血细胞减少症管理的见解。
一名29岁、孕4产0、有1次流产、2次存活分娩史的女性，有自发性细菌性腹膜炎（SBP）病史，继发于AIH的严重全血细胞减少症，在妊娠7周时意外怀孕。尽管多次输血和使用类固醇，但其全血细胞减少症对治疗无反应。孕33周时，因持续性II类胎儿心率监护，她接受了初次剖宫产，娩出一名存活婴儿。分娩时并发出血，需要多种血液制品。产后病程因尿路感染继发败血症、失代偿性肝硬化伴肝性脑病和凝血病而复杂化。胎儿和母亲在分娩后3个月均恢复良好。
本病例突出了妊娠合并AIH管理中的挑战，尤其是治疗严重的无反应性全血细胞减少症的困难，以及在免疫抑制需求与感染风险增加之间取得平衡的困难，感染可能导致诸如SBP和产褥期败血症等后遗症。
