Olewicz-Gawlik Anna, Żeromski Jan, Świerczewska Monika, Idasiak-Piechocka Ilona, Pawlik Magdalena, Sikorska Dorota, Samara Husam, Oko Andrzej, Sikora Jan, Dworacki Grzegorz
Department of Infectious Diseases, Hepatology and Acquired Immune Deficiencies, Poznan University of Medical Sciences, Poland.
Department of Anatomy and Histology, University of Zielona Góra, Poland.
Cent Eur J Immunol. 2019;44(2):210-213. doi: 10.5114/ceji.2019.87074. Epub 2019 Jul 30.
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.
IV型胶原α3和α5链的非胶原（NC1）结构域是抗肾小球基底膜（anti-GBM）病异常免疫反应的主要靶点，该病可通过直接免疫荧光检测到沿肾小球基底膜（GBM）有强烈的线性IgG染色来诊断。肾移植中出现线性GBM固定是一种罕见的发现。我们观察了一名33岁的男性肾移植患者，其出现了新发肾衰竭。对肾脏活检标本的检查显示，在光学显微镜下，有轻度系膜细胞增多，伴有轻度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可见沿毛细血管壁有强烈的线性IgG染色。血清抗GBM抗体为阴性，且未检测到IV型胶原α3和α5链编码NC1结构域的外显子有突变。我们描述了一例肾移植中不典型抗GBM病患者的罕见病例，可能是由影响其自身肾脏的相同过程所致。
