Journal
INTERNAL MEDICINE
Volume 61, Issue 7, Pages 1027-1032Publisher
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.8046-21
Keywords
Rosai-Dorfman disease; IgG4-related disease; tubulointerstitial nephritis
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This article reports a case of extra-nodal RDD presenting as acute tubulointerstitial nephritis mimicking IgG4-RD, and discusses the association between RDD and IgG4-RD.
Rosai-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 IgG4related 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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