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

Intern Med. 2021 Sep 18. doi: 10.2169/internalmedicine.8046-21. Online ahead of print.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.PMID:34544952 | DOI:10.2169/internalmedicine.8046-21
Source: Internal Medicine - Category: Internal Medicine Authors: Source Type: research