Monoclonal immunoglobulin-associated proliferative glomerulonephritis characterized by organized deposits of striated ultra-substructures: A case report
Monoclonal immunoglobulin-associated proliferative glomerulonephritis characterized by organized deposits of striated ultra-substructures: A case report
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DOI：
10.1080/01913123.2017.1336189
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发表时间：
2017-01-01
影响因子：
1
通讯作者：
Joh, Kensuke
中科院分区：
文献类型：
--
作者：
Hara, Shigeo;Tsukaguchi, Hiroyasu;Joh, Kensuke
我们在此报告一个64岁男性的情况下，谁提出了进行性肾小球肾炎值得注意的组织和条纹超微结构。患者入院前3年被诊断为高血压和蛋白尿，随后发展为肾病综合征和肾功能损害。实验室检查没有发现任何感染或自身免疫性疾病的证据。在血清或尿液中未检测到单克隆丙种球蛋白病，但通过流式细胞术检测到少量异常浆细胞克隆。肾活检显示系膜和毛细血管内增生性肾小球肾炎伴小叶加重，伴有局灶性和节段性双轮廓形成。此外，还观察到中度肾小管间质瘢痕和动脉硬化。免疫荧光染色显示IgG、IgM、C3、C1q和纤维蛋白原阳性染色。IgG亚类和轻链染色显示IgG1的有限阳性。电子显微镜检查显示大量的内皮下沉积物，具有分枝状和分支状轮廓。在更高的放大倍数下，在显微镜下观察到周期性条纹图案。肌红蛋白、层粘连蛋白和胶原蛋白（III型和IV型）免疫组化染色呈阴性。类固醇和抗高血压治疗未显示肾功能改善。2年后进行的第二次活检显示类似的小叶增生性肾小球肾炎模式，伴有更广泛的肾小管间质损伤，表明对免疫抑制治疗的反应较差。患者进展为终末期肾病，需要血液透析。我们讨论了在这种情况下观察到的不寻常的子结构的存款的可能来源。
We herein report the case of a 64-year-old male who presented with progressive glomerulonephritis notable for organized and striated ultra-substructures. The patient was diagnosed with hypertension and proteinuria 3 years prior to admission and subsequently developed nephrotic syndrome and impairment of renal function. Laboratory tests did not reveal any evidence of infections or autoimmune diseases. Monoclonal gammopathy was not detected in serum or urine, although a small population of abnormal plasma cell clones was detected by flow cytometry. A renal biopsy showed mesangial and endocapillary proliferative glomerulonephritis with lobular accentuation, accompanied with focal and segmental double-contour formation. Additionally, moderate tubulointerstitial scarring and arteriosclerosis were noted. Immunofluorescence staining revealed positive staining for IgG, IgM, C3, C1q, and fibrinogen. IgG subclass and light chain staining showed restricted positivity for IgG1. Electron microscopy demonstrated massive amounts of subendothelial deposits with a fibrillary and branching profile. At higher magnification, a periodic striated pattern was observed within the microfilament-like structures. Immunohistochemical staining was negative for myoglobin, laminin, and collagens (type III and IV). Steroid and antihypertensive therapy did not show improvement in renal function. The second biopsy performed 2 years later revealed a similar lobular proliferative glomerulonephritis pattern with more extensive tubulointerstitial damage, indicating poor response to immunosuppressive therapy. The patient progressed to end-stage renal disease and required hemodialysis. We discuss the possible origins of the deposits with unusual substructures observed in this case.