Niepolski Leszek, Czekała Anna, Seget-Dubaniewicz Monika, Frydrychowicz Magdalena, Talarska-Markiewicz Patrycja, Kowalska Angelika, Szmelter Jagoda, Salwa-Żurawska Wiesława, Sirek Tomasz, Sobański Dawid, Grabarek Beniamin Oskar, Żurawski Jakub
Department of Physiology, Poznan University of Medical Sciences, 60-567 Poznan, Poland.
Department of Clinical Pathology, Poznan University of Medical Sciences, 60-567 Poznan, Poland.
Biomedicines. 2023 Apr 5;11(4):1101. doi: 10.3390/biomedicines11041101.
C3 glomerulopathies (C3GN) are a group of rare kidney diseases associated with impaired complement regulation. The effects of this disease include the accumulation of complement C3 in the kidneys. Based on the clinical data, as well as light, fluorescence, and electron microscopy results, the diagnoses were verified. The study group consisted of biopsy specimens, which were obtained from 332 patients who were diagnosed with C3 glomerulopathy. In all cases, histopathological examinations were performed; deposits of complement C3 and C1q components, as well as the immunoglobulins IgA, IgG, and IgM, were identified using immunofluorescence. Furthermore, electron microscopy was also performed.
The histopathological examination results presented cases of C3GN (n = 111) and dense deposit disease (DDD; n = 17). The non-classified (NC) group was the most numerous (n = 204). The lack of classification was due to the poor severity of the lesions, even on the electron microscopic examination or in the presence of intense sclerotic lesions.
In cases of suspected C3 glomerulopathies, we believe an electron microscopy examination is necessary. This examination is beneficial in mild-to-extremely-severe cases of this glomerulopathy, where the lesions are barely discernible when using immunofluorescence microscopy.
C3肾小球病（C3GN）是一组与补体调节受损相关的罕见肾脏疾病。该疾病的影响包括补体C3在肾脏中的蓄积。基于临床数据以及光镜、荧光镜和电镜检查结果，进行了诊断验证。研究组由活检标本组成，这些标本取自332例被诊断为C3肾小球病的患者。所有病例均进行了组织病理学检查；使用免疫荧光法鉴定了补体C3和C1q成分以及免疫球蛋白IgA、IgG和IgM的沉积。此外，还进行了电镜检查。
组织病理学检查结果显示有C3GN病例（n = 111）和致密沉积物病（DDD；n = 17）。未分类（NC）组数量最多（n = 204）。缺乏分类是由于病变严重程度较低，即使在电镜检查时或存在严重硬化性病变的情况下也是如此。
在疑似C3肾小球病的病例中，我们认为电镜检查是必要的。该检查对于这种肾小球病的轻度至极重度病例有益，在这些病例中，使用免疫荧光显微镜检查时病变几乎难以辨别。
