A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis

  • Arai Hiroyuki
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan
  • Toda Naohiro
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan
  • Kamimatsuse Ryo
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan Department of Nephrology, Kyoto City Hospital, Japan
  • Nishioka Keisuke
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan Department of Nephrology, Osaka Red Cross Hospital, Japan
  • Endo Shuichiro
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan
  • Akiyama Shinichi
    Division of Nephrology, Department of Internal Medicine, Nagoya University, Graduate School of Medicine, Japan
  • Maruyama Shoichi
    Division of Nephrology, Department of Internal Medicine, Nagoya University, Graduate School of Medicine, Japan
  • Matsubara Takeshi
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan
  • Yokoi Hideki
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan
  • Yanagita Motoko
    Department of Nephrology, Kyoto University, Graduate School of Medicine, Japan

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Abstract

<p>A 58-year-old man with type 1 autoimmune pancreatitis was referred to nephrologists for severe proteinuria. Laboratory data revealed a high serum IgG4 level, hypoalbuminemia, and massive proteinuria, which were compatible with nephrotic syndrome. The renal pathological findings confirmed the diagnosis of secondary membranous nephropathy concurrent with IgG4-related tubulointerstitial nephritis. Despite the improvement of interstitial markers, the proteinuria was refractory to prednisolone, requiring cyclosporine to achieve complete remission. Membranous nephropathy is a rare manifestation of IgG4-related kidney disease. This case shows that the therapeutic response to prednisolone significantly differs between glomerular lesions and interstitial lesions of IgG4-related kidney disease. </p>

Journal

  • Internal Medicine

    Internal Medicine 57 (19), 2873-2877, 2018-10-01

    The Japanese Society of Internal Medicine

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