Division of Nephrology, University of British Columbia, Vancouver, BC, Canada.
St. Paul's Hospital, 1081 Burrard St. Vancouver BC, Vancouver, Canada.
BMC Nephrol. 2018 Jun 28;19(1):152. doi: 10.1186/s12882-018-0949-7.
The diagnosis of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is rare in pregnancy but potentially life threatening. There are no randomized controlled trials to guide the management of AAV in pregnancy and fetal safety data remains limited. Rituximab administration, a treatment for AAV, has been reported in pregnant women with reassuring fetal outcomes in the oncology and rheumatology literature; however, no published reports describe its use in AAV.
We present a case of de novo myeloperoxidase positive (MPO) AAV diagnosed at 22 weeks gestation. Clinical presentation included elevated serum creatinine at 177 μmol/L, hematuria and nephrotic range proteinuria along with high-titre MPO. Diagnosis was confirmed by renal biopsy. Patient was treated with methylprednisolone IV followed by oral prednisone 70 mg daily and Rituximab 650 mg IV weekly for four weeks followed by azathioprine maintenance therapy and prednisone taper. Delivery occurred at 29 weeks gestation via cesarean section for maternal neurologic symptoms concerning for preeclampsia. Maternal and fetal CD + 19 cells were depleted at time of delivery with associated fetal lymphopenia in the absence of infection or other complications related to Rituximab use. The patient experienced a reduction in proteinuria and inflammatory markers following Rituximab therapy; however, serum creatinine increased to 375 μmol/L by 11 weeks post-partum.
We report the first use, to our knowledge, of Rituximab with corticosteroids for induction therapy of AAV in pregnancy.
抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)在妊娠中罕见,但可能危及生命。目前尚无随机对照试验来指导妊娠中 AAV 的治疗,胎儿安全性数据仍然有限。利妥昔单抗是治疗 AAV 的一种药物,在肿瘤学和风湿病学文献中已有报道在妊娠妇女中使用,具有令人安心的胎儿结局;然而,尚无文献报道其在 AAV 中的应用。
我们报告了一例新诊断的髓过氧化物酶阳性(MPO)AAV 病例,该病例发生在妊娠 22 周时。临床表现包括血清肌酐升高至 177 μmol/L,血尿和肾病范围蛋白尿,以及高滴度 MPO。通过肾活检确诊。患者接受了甲基泼尼松龙 IV 治疗,随后口服泼尼松每日 70mg,利妥昔单抗每周 650mg IV 共四周,随后接受硫唑嘌呤维持治疗和泼尼松减量。由于母亲出现子痫前期相关的神经系统症状,患者于妊娠 29 周行剖宫产分娩。在分娩时,母亲和胎儿的 CD+19 细胞被耗尽,同时胎儿出现淋巴细胞减少,但没有感染或与利妥昔单抗使用相关的其他并发症。患者在接受利妥昔单抗治疗后蛋白尿和炎症标志物减少;然而,在产后 11 周时血清肌酐增加至 375 μmol/L。
我们报告了首例在妊娠中使用利妥昔单抗联合皮质类固醇治疗 AAV 的病例。