Open Access Case report

IgG4-related tubulointerstitial nephritis accompanied with cystic formation

Hideo Fukuhara1, Yoshinori Taniguchi2, Manabu Matsumoto3, Naoto Kuroda4, Satoshi Fukata1, Keiji Inoue1*, Shimpei Fujimoto2, Yoshio Terada2 and Taro Shuin1

Author Affiliations

1 Department of Urology, Kochi Medical School, Kohasu, Oko, Nankoku, Kochi 783-8505, Japan

2 Department of Endocrinology, Metabolism and Nephrology, Kochi, Japan

3 Department of Pathology, Kochi Medical School, Kochi, Japan

4 Department of Diagnostic Pathology, Kochi Red Cross Hospital, Kochi, Japan

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BMC Urology 2014, 14:54  doi:10.1186/1471-2490-14-54

Published: 20 July 2014



An immunoglobulin G4 (IgG4)-related disease is important disease in differential diagnosis of tumors in kidney, pancreas, lung and other organs. The imaging findings of IgG4-related kidney diseases are usually expressed as defect contrast region, while cystic formation in kidney is extremely rare. Here, we report a case of IgG4-related tubulointerstitial nephritis with renal cystic change caused by the narrowing or obstruction of collecting duct in renal medulla.

Case presentation

Abdominal contrasted CT scan showed a 31 × 24 mm cystic tumor at the upper pole of the right kidney and multiple low-attenuation areas in the left kidney. 18 F-fluorodeoxyglucose (FDG)-PET/CT scan showed moderate FDG accumulation of cystic tumor in marginal lesion. In addition, FDG-PET/CT scan also showed moderate FDG accumulation in the pancreatic body. Laparoscopic right nephrectomy was performed. Histological examination was revealed lymphoplasmacytic infiltrate with focal fibrosis and severe narrowing or obstruction of lumen of collecting duct in renal medulla. Furthermore, the IgG4 positive plasma cells infiltrated exceeding 10 cells per one high-power field in renal medulla. The ratio of IgG4-plasma cells to IgG-positive plasma cells was about 50%. The serum level of IgG4 was also elevated (218 mg/dl). Based on these findings, we finally diagnosed IgG4-related tubulointerstitial nephritis with renal cystic change.


IgG4-related kidney disease might cause cystic formation by severe narrowing and obstruction of collecting duct.

IgG4-related tubulointerstitial nephritis; Renal cyst change; Collecting duct