Tatsuhito Miyanaga1, Keishi Mizuguchi2, Satoshi Hara3, Takeshi Zoshima1, Dai Inoue4, Ryo Nishioka1, Ichiro Mizushima1, Kiyoaki Ito1, Hiroshi Fuji1, Kazunori Yamada5, Yuki Sato6,7, Motoko Yanagita7,8, Mitsuhiro Kawano1. 1. Division of Rheumatology, Department of Internal Medicine, 13-1 Takaramachi, Kanazawa, Ishikawa, Japan. 2. Department of Diagnostic Pathology, Kanazawa University Hospital, 13-1 Takaramachi, Kanazawa, Ishikawa, Japan. 3. Division of Rheumatology, Department of Internal Medicine, 13-1 Takaramachi, Kanazawa, Ishikawa, Japan. satoshihara@staff.kanazawa-u.ac.jp. 4. Department of Radiology, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa, Ishikawa, Japan. 5. Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, Japan. 6. Department of Nephrology, Kyoto University Graduate School of Medicine, Yoshidakonoe-cho, Sakyo-ku, Kyoto, Japan. 7. Medical Innovation Center TMK Project, Graduate School of Medicine, Kyoto University, 53 Shogoin, Kawahara-cho, Sakyo-ku, Kyoto, Japan. 8. Institute for the Advanced Study of Human Biology (ASHBi), Kyoto University, Yoshida-honmachi, Sakyo-ku, Kyoto, Japan.
Abstract
BACKGROUND: IgG4-related kidney disease causes renal impairment of unknown pathogenesis that may progress to kidney failure. Although ectopic germinal centers contribute to the pathogenesis of the head and neck lesions of IgG4-related disease, the presence of tertiary lymphoid tissue (TLT) containing germinal centers in IgG4-RKD has rarely been reported. CASE PRESENTATION: We report a 72-year-old Japanese man who had IgG4-related tubulointerstitial nephritis (TIN) with TLT formation incidentally detected in a resected kidney with mass lesion of IgG4-related ureteritis in the ureteropelvic junction. During follow-up for past surgical resection of a bladder tumor, renal dysfunction developed and a ureter mass was found in the right ureteropelvic junction, which was treated by nephroureterectomy after chemotherapy. Pathology revealed no malignancy but abundant IgG4-positive cell infiltration, obliterative phlebitis and storiform fibrosis, confirming the diagnosis of IgG4-related ureteritis. In the resected right kidney, lymphoplasmacytes infiltrated the interstitium with focal distribution in the renal subcapsule and around medium vessels without storiform fibrosis, suggesting the very early stage of IgG4-TIN. Lymphocyte aggregates were also detected at these sites and consisted of B, T, and follicular dendritic cells, indicating TLT formation. IgG4-positive cells infiltrated around TLTs. CONCLUSIONS: Our case suggests that TLT formation is related with the development of IgG4-TIN and our analysis of distribution of TLT have possibility to elucidate IgG4-TIN pathophysiology.
BACKGROUND: IgG4-related kidney disease causes renal impairment of unknown pathogenesis that may progress to kidney failure. Although ectopic germinal centers contribute to the pathogenesis of the head and neck lesions of IgG4-related disease, the presence of tertiary lymphoid tissue (TLT) containing germinal centers in IgG4-RKD has rarely been reported. CASE PRESENTATION: We report a 72-year-old Japanese man who had IgG4-related tubulointerstitial nephritis (TIN) with TLT formation incidentally detected in a resected kidney with mass lesion of IgG4-related ureteritis in the ureteropelvic junction. During follow-up for past surgical resection of a bladder tumor, renal dysfunction developed and a ureter mass was found in the right ureteropelvic junction, which was treated by nephroureterectomy after chemotherapy. Pathology revealed no malignancy but abundant IgG4-positive cell infiltration, obliterative phlebitis and storiform fibrosis, confirming the diagnosis of IgG4-related ureteritis. In the resected right kidney, lymphoplasmacytes infiltrated the interstitium with focal distribution in the renal subcapsule and around medium vessels without storiform fibrosis, suggesting the very early stage of IgG4-TIN. Lymphocyte aggregates were also detected at these sites and consisted of B, T, and follicular dendritic cells, indicating TLT formation. IgG4-positive cells infiltrated around TLTs. CONCLUSIONS: Our case suggests that TLT formation is related with the development of IgG4-TIN and our analysis of distribution of TLT have possibility to elucidate IgG4-TIN pathophysiology.
Authors: Hyon K Choi; John H Stone; Zachary S Wallace; Yuqing Zhang; Cory A Perugino; Ray Naden Journal: Ann Rheum Dis Date: 2019-01-05 Impact factor: 19.103