Hinchingbrooke Health Care NHS Trust, Huntingdon, UK.
Clin Exp Rheumatol. 2009 Jan-Feb;27(1 Suppl 52):S86-8.
Wegener's granulomatosis (WG) is rarely diagnosed during the reproductive years and uncommonly manifests for the first time during pregnancy. We report a case of de novo WG presenting at 30 weeks gestation with classical symptoms of WG (ENT, pulmonary). The diagnosis was confirmed by radiological, laboratory, and histological investigations. With a multidisciplinary approach, she had a successful vaginal delivery of a healthy baby. She was treated successfully by a combination of steroids, azathioprine and intravenous immunoglobulin in the active phase of disease for induction of remission and by azathioprine and steroids for maintenance of remission. The significant improvement in her symptoms allowed us to continue her pregnancy to 37 weeks when delivery was electively induced. Transplacental transmission of PR3-ANCA occurred but the neonate remained well. This case of de novo WG during pregnancy highlights the seriousness of this disease and the challenge in management of such patients.
韦格纳肉芽肿病（WG）在生育期很少被诊断，并且在妊娠期间首次出现的情况也不常见。我们报告了一例新诊断的 WG 病例，该病例在妊娠 30 周时出现了经典的 WG 症状（耳鼻喉、肺部）。通过影像学、实验室和组织学检查确诊。通过多学科方法，她成功地进行了阴道分娩，产下了一个健康的婴儿。在疾病的活动期，她接受了类固醇、硫唑嘌呤和静脉注射免疫球蛋白的联合治疗以诱导缓解，随后接受了硫唑嘌呤和类固醇治疗以维持缓解，治疗取得了成功。她的症状显著改善，使我们能够将她的妊娠继续到 37 周，然后择期引产。PR3-ANCA 通过胎盘传播，但新生儿情况良好。这例妊娠期间新发的 WG 病例突出了该疾病的严重性以及对这类患者进行管理的挑战。
