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抗肾小球基底膜病合并膜性肾病的临床和病理特征:一项观察性研究。

Clinical and pathological features of anti-glomerular basement membrane disease associated with membranous nephropathy: an observational study.

机构信息

Department of Nephrology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.

Department of Nephrology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.

出版信息

Ren Fail. 2022 Dec;44(1):1904-1914. doi: 10.1080/0886022X.2022.2141645.

DOI:10.1080/0886022X.2022.2141645
PMID:36351876
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9662048/
Abstract

To investigate the clinical manifestations, pathological features, pathogenesis, treatment, and prognosis of anti-glomerular basement membrane (anti-GBM) disease with membranous nephropathy (MN). Seven patients with anti-GBM disease and concurrent MN were enrolled in this study. Control subjects included 13 patients with anti-GBM glomerulonephritis (GN) and 6 with anti-GBM disease and concurrent anti-neutrophil cytoplasmic antibodies-associated disease (anti-GBM + ANCA). Laboratory tests and pathological information were analyzed before immunosuppressive therapy or plasmapheresis administration. Prognosis was assessed in continuous follow-up. In the anti-GBM + MN group, 28.57% of patients exhibited acute kidney disease, lower than that in the anti-GBM GN group (84.62%,  = .022). None of the anti-GBM + MN or + ANCA patients exhibited hemoptysis, but 15.4% of anti-GBM GN patients did, with no significant difference ( = .720). Only 14.3% of anti-GBM + MN patients had crescentic GN. The proportion of necrosis averaged 29.0% in the anti-GBM + MN group. Survival curve analysis revealed that renal outcomes in the anti-GBM + MN group were better than those in the anti-GBM GN group ( = .019). Patients with both anti-GBM disease and MN showed atypical anti-GBM GN. They had a lower proportion of glomerular crescents and a better renal function prognosis than patients with classical anti-GBM GN. To improve renal recovery, early identification and treatment of anti-GBM disease associated with MN is needed.

摘要

探讨合并膜性肾病(MN)的抗肾小球基底膜(anti-GBM)病的临床表现、病理特征、发病机制、治疗和预后。本研究纳入了 7 例合并 MN 的抗-GBM 病患者。对照组包括 13 例抗-GBM 肾小球肾炎(GN)患者和 6 例合并抗-GBM 病和抗中性粒细胞胞质抗体相关疾病(anti-GBM+ANCA)患者。在进行免疫抑制治疗或血浆置换前,分析实验室检查和病理信息。通过连续随访评估预后。在 anti-GBM+MN 组中,28.57%的患者表现为急性肾损伤,低于 anti-GBM GN 组(84.62%,=0.022)。anti-GBM+MN 或+ANCA 组均无患者出现咯血,但 15.4%的 anti-GBM GN 患者出现咯血,差异无统计学意义(=0.720)。仅 14.3%的 anti-GBM+MN 患者存在新月体性 GN。anti-GBM+MN 组坏死的平均比例为 29.0%。生存曲线分析显示,anti-GBM+MN 组的肾脏结局优于 anti-GBM GN 组(=0.019)。同时患有抗-GBM 病和 MN 的患者表现为非典型抗-GBM GN。与经典抗-GBM GN 患者相比,他们的肾小球新月体比例较低,肾功能预后较好。为改善肾恢复,需要早期识别和治疗与 MN 相关的抗-GBM 病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ddf2/9662048/52e1c6ec21bf/IRNF_A_2141645_F0002_C.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ddf2/9662048/9c6ac40e3dec/IRNF_A_2141645_F0001_B.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ddf2/9662048/52e1c6ec21bf/IRNF_A_2141645_F0002_C.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ddf2/9662048/9c6ac40e3dec/IRNF_A_2141645_F0001_B.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ddf2/9662048/52e1c6ec21bf/IRNF_A_2141645_F0002_C.jpg

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