A possible role of classical complement pathway activation in the pathogenesis of immunoglobulin G nephropathy: a case report.

Momoko Arai, Akiko Mii, Akio Hirama, Tomohiro Kaneko, Tetsuya Kashiwagi, Akira Shimizu, Yukinao Sakai

CEN case reports 2023 Feb

filter terms:

Immunoglobulin G (IgG) nephropathy refers to a rare group of diseases characterized by deposits of IgG in the mesangial region. However, IgG nephropathy is controversial as a single disease entity, and its pathogenesis remains to be elucidated. In the present report, we discuss a case of IgG nephropathy in which we observed activation of the classical complement pathway.A 47-year-old woman was admitted to our hospital with nephrotic syndrome. Light-microscopic examination revealed neither proliferative nor sclerotic lesions in the glomeruli. However, unusual and large deposits were observed in the paramesangial area. An immunofluorescence study revealed predominant IgG and C1q and slight C3 deposits in the paramesangial area, suggesting immune-complex-type glomerular disease. An electron microscopic study also revealed different sizes of non-organized electron-dense deposits with a similar pattern of distribution, which were accompanied by foot process effacement. Clinically, there was no evidence of systemic diseases, such as infectious or autoimmune diseases (including systemic lupus erythematosus). Based on these findings, she was diagnosed with IgG nephropathy and treated with prednisolone. Steroid therapy was effective, and complete remission was maintained.Additional immunological examination revealed that IgG deposits were polyclonal and consisted mainly of the IgG1 and IgG3 subclasses. Furthermore, staining was positive for C4d and C5b-9. The present findings indicate that the pathogenesis of IgG nephropathy in our patient may have involved activation of the classical complement pathway. © 2022. The Author(s) under exclusive licence to The Japan Society of Nephrology.

Citation

Momoko Arai, Akiko Mii, Akio Hirama, Tomohiro Kaneko, Tetsuya Kashiwagi, Akira Shimizu, Yukinao Sakai. A possible role of classical complement pathway activation in the pathogenesis of immunoglobulin G nephropathy: a case report. CEN case reports. 2023 Feb;12(1):14-22