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

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Abstract

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.

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Availability of data and materials

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Acknowledgements

We thank Ms. Arimi Ishikawa and Ms. Asae Kurosawa for their technical assistance.

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Authors and Affiliations

  1. Department of Nephrology, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, Tokyo, 113-8602, Japan

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

  2. Analytic Human Pathology, Nippon Medical School, Tokyo, Japan

    Akira Shimizu

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  1. Momoko Arai
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  2. Akiko Mii
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  3. Akio Hirama
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  4. Tomohiro Kaneko
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  5. Tetsuya Kashiwagi
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  6. Akira Shimizu
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  7. Yukinao Sakai
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Contributions

MA, HA, TK, AM, and YS treated the patient and evaluated the clinical images. AM and AS were responsible for the pathological diagnosis. MA, AM, and TK contributed to clinical data acquisition. MA, AM, and AS interpreted the data and prepared the manuscript. All authors read and approved the final version of this manuscript.

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Correspondence to Akiko Mii.

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The authors have declared that no conflict of interest exists.

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No experimental study was conducted. All procedures performed in this case were in accordance with the ethical standards of the institutional committee and with the 1975 Helsinki declaration and its later amendments or comparable ethical standards.

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Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. This work does not contain any studies with animals performed by any of the authors.

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Arai, M., Mii, A., Hirama, A. et al. A possible role of classical complement pathway activation in the pathogenesis of immunoglobulin G nephropathy: a case report. CEN Case Rep 12, 14–22 (2023). https://doi.org/10.1007/s13730-022-00710-5

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  • DOI: https://doi.org/10.1007/s13730-022-00710-5

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