Al-Rabadi Laith, Ayalon Rivka, Bonegio Ramon G, Ballard Jennifer E, Fujii Alan M, Henderson Joel M, Salant David J, Beck Laurence H
Department of Medicine, Renal Section, Boston University Medical Center, Boston, MA.
Department of Obstetrics and Gynecology, Boston University Medical Center, Boston, MA.
Am J Kidney Dis. 2016 May;67(5):775-8. doi: 10.1053/j.ajkd.2015.10.031. Epub 2015 Dec 29.
There is little information about pregnancy outcomes in patients with active membranous nephropathy (MN), especially those with circulating autoantibodies to M-type phospholipase A₂receptor (PLA₂R), the major autoantigen in primary MN. We present what we believe to be the first known case of successful pregnancy in a 39-year-old woman with PLA₂R-associated MN. In the year prior to pregnancy, the patient developed anasarca, hypoalbuminemia (albumin, 1.3-2.2g/dL), and proteinuria (protein excretion, 29.2 g/d). Kidney biopsy revealed MN with staining for PLA₂R, and the patient was seropositive for anti-PLA₂R autoantibodies. She did not respond to conservative therapy and was treated with intravenous rituximab (2 doses of 1 g each). Several weeks after presentation, she was found to be 6 weeks pregnant and was closely followed up without further immunosuppressive treatment. Proteinuria remained with protein excretion in the 8- to 12-g/d range. Circulating anti-PLA₂R levels declined but were still detectable. At 38 weeks, a healthy baby girl was born, without proteinuria at birth or at her subsequent 6-month postnatal visit. At the time of delivery, the mother still had detectable circulating anti-PLA₂R of immunoglobulin G1 (IgG1), IgG3, and IgG4 subclasses, although at low titers. Only trace amounts of IgG4 anti-PLA₂R were found in cord blood. Potential reasons for the discrepancy between anti-PLA₂R levels in the maternal and fetal circulation are discussed.
关于活动性膜性肾病(MN)患者的妊娠结局,尤其是那些对M型磷脂酶A₂受体(PLA₂R)(原发性MN的主要自身抗原)具有循环自身抗体的患者,相关信息很少。我们报告了我们认为是首例已知的成功妊娠病例,该患者为一名39岁患有PLA₂R相关MN的女性。在妊娠前一年,患者出现全身性水肿、低白蛋白血症(白蛋白,1.3 - 2.2g/dL)和蛋白尿(蛋白排泄量,29.2g/d)。肾活检显示为PLA₂R染色阳性的MN,且患者抗PLA₂R自身抗体血清学呈阳性。她对保守治疗无反应,接受了静脉注射利妥昔单抗治疗(2剂,每剂1g)。就诊几周后,发现她已怀孕6周,随后在未进行进一步免疫抑制治疗的情况下接受密切随访。蛋白尿持续存在,蛋白排泄量在8至12g/d范围内。循环抗PLA₂R水平下降但仍可检测到。38周时,一名健康女婴出生,出生时及随后6个月的产后随访均无蛋白尿。分娩时,母亲循环中的免疫球蛋白G1(IgG1)、IgG3和IgG4亚类抗PLA₂R仍可检测到,尽管滴度较低。脐血中仅发现微量的IgG4抗PLA₂R。文中讨论了母胎循环中抗PLA₂R水平存在差异的潜在原因。