Ounhasuttiyanon Areerat, Tantranont Ngoentra, Srithongkul Thatsaphan
Division of Nephrology, Department of Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, Salaya, Thailand.
Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Salaya, Thailand.
Case Rep Nephrol. 2023 Nov 11;2023:6963543. doi: 10.1155/2023/6963543. eCollection 2023.
Antiglomerular basement membrane disease (anti-GBM) is an unusual cause of glomerulonephritis. Patients usually present with rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. The diagnosis is based on linear deposits of IgG along the GBM and the presence of anti-GBM antibodies. However, cases with atypical anti-GBM disease in which an anti-GBM antibody was not detected have been reported. We report a 29-year-old pregnant woman with underlying systemic lupus erythematosus (SLE) who presented with severe glomerulonephritis due to atypical antiglomerular basement membrane disease. She was initially diagnosed with active lupus nephritis and her renal function gradually worsened after steroid treatment, so the pregnancy was terminated due to the high maternal and fetal risks. A kidney biopsy showed linear capillary wall staining with fibrous crescents without endocapillary proliferation. The anti-GBM antibody showed negative results two times, so she was diagnosed with atypical anti-GBM disease. Treatment began with intravenous pulse methylprednisolone and continued with mycophenolate mofetil and prednisolone. Due to the intolerability of side effects, the treatment regimen was subsequently changed to intravenous cyclophosphamide. Although she had a significant improvement in clinical edema, serum albumin, and hematuria, her renal function gradually decreased during the 12 months of treatment. A review of the literature showed that the atypical anti-GBM is less aggressive than the typical anti-GBM disease. However, several patients had persistent renal dysfunction and 20-30% of patients had progression to ERSD. To the best of our knowledge, this is the first case of atypical anti-GBM disease in pregnant patients with suspected SLE reported in the literature.
抗肾小球基底膜病（anti-GBM）是肾小球肾炎的一种罕见病因。患者通常表现为快速进展性肾小球肾炎，可伴有或不伴有肺出血。诊断基于IgG沿肾小球基底膜的线性沉积以及抗GBM抗体的存在。然而，已有报道称存在非典型抗GBM病病例，其中未检测到抗GBM抗体。我们报告一例29岁患有系统性红斑狼疮（SLE）的孕妇，因非典型抗肾小球基底膜病出现严重肾小球肾炎。她最初被诊断为活动性狼疮性肾炎，激素治疗后肾功能逐渐恶化，由于母婴风险高，终止了妊娠。肾活检显示毛细血管壁呈线性染色，伴有纤维性新月体形成，无毛细血管内增生。抗GBM抗体两次检测均为阴性，因此她被诊断为非典型抗GBM病。治疗开始采用静脉注射甲泼尼龙冲击治疗，随后继续使用霉酚酸酯和泼尼松龙。由于副作用难以耐受，随后治疗方案改为静脉注射环磷酰胺。尽管她的临床水肿、血清白蛋白和血尿有显著改善，但在治疗的12个月期间肾功能逐渐下降。文献回顾显示，非典型抗GBM病的侵袭性低于典型抗GBM病。然而，有几位患者存在持续性肾功能不全，20%至30%的患者进展为终末期肾病（ERSD）。据我们所知，这是文献中报道的首例疑似SLE的孕妇非典型抗GBM病病例。
