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. 2023 Apr 5;11(4):1101.
doi: 10.3390/biomedicines11041101.

Diagnostic Problems in C3 Glomerulopathy

Leszek Niepolski  1 , Anna Czekała  2 , Monika Seget-Dubaniewicz  2 , Magdalena Frydrychowicz  3 , Patrycja Talarska-Markiewicz  4 , Angelika Kowalska  4 , Jagoda Szmelter  4 , Wiesława Salwa-Żurawska  2 , Tomasz Sirek  5   6 , Dawid Sobański  6   7 , Beniamin Oskar Grabarek  6   7 , Jakub Żurawski  4
Affiliations

Diagnostic Problems in C3 Glomerulopathy

Leszek Niepolski et al. Biomedicines. .

Abstract

Background: 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.

Results: 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.

Conclusions: 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.

Keywords: C3 glomerulonephritis (C3GN); C3 glomerulopathy; clinical manifestation; dense deposit disease (DDD); electron microscopy; kidney biopsy.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Diagram of the study flow. G3GN, C3 glomerulonephritis; DDD, dense deposit disease; NC, non-classified group; C3, C1q, complement component; IgA, immunoglobulin A; IgG, immunoglobulin g; IgM, immunoglobulin M.
Figure 2
Figure 2
Age distribution in the study group.
Figure 3
Figure 3
Number of cases in each group diagnosed with a particular symptom or symptoms.
Figure 4
Figure 4
Kidney glomerulus with an increased number of cells and a well-defined lobular structure. H&E, 100× magnification.
Figure 5
Figure 5
Double-contouring of the walls of the capillary loops. Impregnation with silver-containing salt according to a Jones stain, 400× magnification.
Figure 6
Figure 6
A case of C3GN. Electron microscopic image of the glomerulus, with transposition of mesangial cell protuberances to the periphery of capillary loops and with a ‘double’ basement membrane. Subendothelial deposits. Electron microscopy, 8750× magnification.
Figure 7
Figure 7
The case of C3GN. Electron microscopic image of the glomerulus with transposition of mesangial cell protrusions to the periphery of the capillary loop and presence of subepithelial deposits. Electron microscopy, 8750× magnification. Mes, mesangium; EP, epithelium; END, endothelium; D, deposits.
Figure 8
Figure 8
A case of DDD. Electron microscopic image of a glomerulus with a markedly thickened basement membrane structure, as well as a transposition of mesangial cell protrusions. Electron microscopy, 7500× magnification. MB, basement membrane; Mes, mesangium.
See this image and copyright information in PMC

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