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Emergence of proteinase 3-antineutrophil cytoplasmic antibody-associated glomerulonephritis with mesangial immune deposition during the clinical course of IgG λ monoclonal gammopathy of uncertain significance

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Abstract

Patients with monoclonal gammopathy of uncertain significance (MGUS) is sometimes associated with renal diseases, usually due to the deposition of secreted monoclonal immunoglobulin or a fragment thereof, a condition which is defined as monoclonal gammopathy of renal significance. Patients with MGUS appear to be at increased risk for various autoimmune conditions. We report the case of a 68-year-old man developed nephritic syndrome and mild renal insufficiency during the course of IgG λ MGUS. Laboratory findings showed hypocomplementemia, cryoglobulinemia, proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) positivity and monoclonal proteins (λ light chain and λ-Bence-Jones protein) in the urine. A kidney biopsy revealed crescentic glomerulonephritis with mesangial immune deposits without paraproteins. Treatment with prednisolone for ANCA-associated glomerulonephritis, normalized urinalysis and decreased PR3-ANCA but MGUS persisted. This is a rare case of PR3-ANCA-associated glomerulonephritis with comorbid IgG λ MGUS with various pathological paraproteins. We highlight it as a clinical example with diagnostic and therapeutic implications.

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Acknowledgements

We would like to thank Ms. Hiromi Yamaguchi for her technical assistance.

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Authors and Affiliations

  1. Department of Internal Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, Japan

    Masaki Ueno, Shinichiro Asakawa, Shigeyuki Arai, Michito Nagura, Osamu Yamazaki, Yoshifuru Tamura, Shigeru Shibata & Yoshihide Fujigaki

  2. Division of Nephrology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan

    Sawako Kobayashi

  3. Department of Integrated Diagnostic Pathology, Nippon Medical School, Bunkyo-ku, Tokyo, Japan

    Ryuji Ohashi

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  1. Masaki Ueno
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  2. Sawako Kobayashi
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  3. Shinichiro Asakawa
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  4. Shigeyuki Arai
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Correspondence to Yoshihide Fujigaki.

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Ueno, M., Kobayashi, S., Asakawa, S. et al. Emergence of proteinase 3-antineutrophil cytoplasmic antibody-associated glomerulonephritis with mesangial immune deposition during the clinical course of IgG λ monoclonal gammopathy of uncertain significance. CEN Case Rep 11, 463–470 (2022). https://doi.org/10.1007/s13730-022-00703-4

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