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An enigmatic case of IgG4-related nephropathy and an updated review of the literature

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Abstract

IgG4-related disease (IgG4-RD) is still an underestimated disorder which affects multiple organs, and its recognition as a distinct clinical disease has been only proved in the recent decades. The renal involvement has been documented in approximately 15% of patients with IgG4-RD, and the typical manifestation is a tubulo-interstitial nephritis. The main histological findings in IgG4-RD are typically a dense tissue infiltration of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis, and frequently elevated IgG4 serum levels. Herein we report our atypical and peculiar clinical presentation of an IgG4-related nephropathy (IgG4-RN) and the remarkable response to rituximab (RTX) treatment at the renal imaging with computerized tomography assessment. The current nephrological evidences support the renal function recovery after steroids or steroids plus RTX therapy, even if the renal imaging data are not always shown. In a complex and enigmatic clinical scenario such as the IgG4-RN, both the renal biopsy and the renal imaging before and after the immunosuppressive therapy become mandatory tools to thoroughly define the diagnosis, the management and the response to the immunological therapy.

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The authors have no relevant financial or non-financial interests to disclose, and no funding was received to assist with the preparation of this manuscript.

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Authors and Affiliations

  1. Division of Nephrology, Dialysis and Renal Transplantation, ASST Grande Ospedale Metropolitano Niguarda, 20162, Milan, Italy

    Leonardo Spatola, Federica Ravera, Maria Chiara Sghirlanzoni, Simona Verdesca, Alberto Menegotto, Marialuisa Querques, Valeriana Colombo & Enrico Eugenio Minetti

  2. Division of Pathology, ASST Grande Ospedale Territoriale Niguarda, 20162, Milan, Italy

    Mario Livio Camozzi

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  1. Leonardo Spatola
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Correspondence to Leonardo Spatola.

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Spatola, L., Ravera, F., Sghirlanzoni, M.C. et al. An enigmatic case of IgG4-related nephropathy and an updated review of the literature. Clin Exp Med 21, 493–500 (2021). https://doi.org/10.1007/s10238-021-00696-x

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