• Internal medicine · Apr 2022

    Case Reports

    Acute Tubulointerstitial Nephritis in Rosai-Dorfman Disease Mimicking IgG4-related Disease.

    • Satoshi Kurahashi, Naohiro Toda, Masaaki Fujita, Katsuya Tanigaki, Jun Takeoka, Hisako Hirashima, Eri Muso, Katsuhiro Io, Takaki Sakurai, and Toshiyuki Komiya.
    • Department of Nephrology, Kansai Electric Power Hospital, Japan.
    • Intern. Med. 2022 Apr 1; 61 (7): 1027-1032.

    AbstractRosai-Dorfman-Destombes disease (RDD) is a non-Langerhans cell histiocytosis characterized by the accumulation of histiocytes inside the lymph nodes or extranodally. The association between RDD and IgG4-related disease (IgG4-RD) is discussed. We herein report a case of RDD manifesting as acute tubulointerstitial nephritis mimicking IgG4-RD. The first renal biopsy showed severe tubulointerstitial nephritis with infiltration of S100-positive histiocytes and IgG4-positive plasma cells; storiform fibrosis and obliterative phlebitis were not confirmed. After prednisolone therapy, IgG4-positive cells and S100-positive histiocytes were decreased, but the IgG4/IgG ratio increased despite clinical improvement. These findings indicated extranodal RDD in the kidney presenting as tubulointerstitial nephritis.

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