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A case of bizarre posttransplant anti-glomerular basement membrane disease.
Central European Journal of Immunology ( IF 1.5 ) Pub Date : 2019-09-19 , DOI: 10.5114/ceji.2019.87074
Anna Olewicz-Gawlik 1, 2 , Jan Żeromski 3 , Monika Świerczewska 4 , Ilona Idasiak-Piechocka 5 , Magdalena Pawlik 5 , Dorota Sikorska 6 , Husam Samara 3 , Andrzej Oko 5 , Jan Sikora 3 , Grzegorz Dworacki 3
Affiliation  

The non-collagenous (NC1) domain of α3 and α5 chains of type IV collagen are eminent targets of abnormal immune response in anti-glomerular basement membrane (anti-GBM) disease, which can be diagnosed by the presence of strong linear IgG staining along GBM detected by direct immunofluorescence. The presence of linear GBM fixation in renal allograft is a rare finding. We observed a 33-year-old male with de novo renal failure in a kidney transplant. An examination of a kidney biopsy specimen revealed, in light microscopy, mild mesangial hypercellularity together with mild focal interstitial fibrosis and sparse inflammatory infiltrate. In immunofluorescence microscopy strong linear IgG staining along the capillary walls was seen. Serum anti-GBM antibodies were negative and no mutation in exons coding NC1 domains of α3 and α5 chains of type IV collagen were detected. We described a rare case of a patient with atypical anti-GBM disease in renal allograft, caused probably by the same process which affected the native kidneys.

中文翻译:

一例怪异的移植后抗肾小球基底膜病。

IV型胶原的α3和α5链的非胶原(NC1)结构域是抗肾小球基底膜(anti-GBM)疾病中异常免疫反应的主要靶标,可以通过沿其上存在强烈的线性IgG染色来诊断通过直接免疫荧光检测GBM。肾同种异体移植中线性GBM固定的存在是一个罕见的发现。我们在肾脏移植中观察到一名33岁的男性患有从新肾功能衰竭。肾脏活检标本的检查显示,在光学显微镜下可见轻度的肾小球系膜细胞增生,以及轻度的局灶性间质纤维化和稀疏的炎性浸润。在免疫荧光显微镜下,沿毛细管壁观察到强线性IgG染色。血清抗GBM抗体为阴性,未检测到编码IV型胶原α3和α5链的NC1结构域的外显子突变。我们描述了罕见的肾脏异体移植中非典型抗GBM疾病患者的病例,可能是由影响天然肾脏的相同过程引起的。
更新日期:2019-11-01
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