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A case of minimal change nephrotic syndrome complicated by Kimura disease treated with rituximab

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Abstract

Kimura disease (eosinophilic granuloma of the soft tissue) is a benign granulomatous disease complicated by nephrotic syndrome. Herein, we report a case of recurrent minimal change nephrotic syndrome (MCNS) complicated by Kimura disease that was successfully treated with rituximab. A 57-year-old man presented to our hospital with relapsed nephrotic syndrome with worsening swelling of the right anterior ear and elevated serum IgE. MCNS was diagnosed on renal biopsy. Treatment with 50 mg of prednisolone rapidly placed the patient in remission. Therefore, RTX 375 mg/m2 was added to the treatment regimen, and steroid therapy was tapered. Early steroid tapering was successful, and the patient is currently in remission. In this case, the nephrotic syndrome flare-up was accompanied by worsening Kimura disease. Rituximab reduced the worsening of symptoms related to Kimura disease, including head and neck lymphadenopathy and elevated IgE levels. Kimura disease and MCNS may share a common IgE-mediated type I allergic condition. Rituximab effectively treats these conditions. In addition, rituximab suppresses Kimura disease activity in patients with MCNS, enables early tapering of steroids, and reduces the total dose of steroids.

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Authors and Affiliations

  1. Department of Nephrology, Keiyu Hospital, 3-7-3 Minatomirai, Nishi-Ku, Yokohama, Kanagawa, 220-8521, Japan

    Ryuto Yoshida, Yoshikazu Hara, Yoshiaki Fujii & Hiroto Matuda

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  1. Ryuto Yoshida
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  2. Yoshikazu Hara
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  3. Yoshiaki Fujii
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  4. Hiroto Matuda
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Correspondence to Ryuto Yoshida.

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All the authors have declared no competing interest.

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The procedures performed in this study that involve human participants were performed in accordance with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards and the ethical standards of the institutional and national research committee where the studies were conducted.

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Informed consent was obtained from all patients included in this study. Additional informed consent was obtained from all patients with identifying information included in this study.

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Yoshida, R., Hara, Y., Fujii, Y. et al. A case of minimal change nephrotic syndrome complicated by Kimura disease treated with rituximab. CEN Case Rep 12, 368–373 (2023). https://doi.org/10.1007/s13730-023-00778-7

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  • DOI: https://doi.org/10.1007/s13730-023-00778-7

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