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01.12.2015 | Case report | Ausgabe 1/2015 Open Access

BMC Urology 1/2015

IgG4-related kidney disease from the renal pelvis that mimicked urothelial carcinoma: a case report

BMC Urology > Ausgabe 1/2015
Hui Zhang, Xinyu Ren, Wen Zhang, Di Yang, Ruie Feng
Wichtige Hinweise
Hui Zhang and Xinyu Ren contributed equally to this work.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

HZ and XYR drafted the manuscript and participated in the final diagnosis. WZ, DY, and REF critically revised the manuscript for important intellectual content and gave final approval of the version to be published. The final manuscript was read and approved by all authors.



IgG4-related kidney disease is a comprehensive term for renal lesions associated with IgG4-related disease, which mainly manifests as plasma cell-rich tubulointerstitial nephritis with increased IgG4+ plasma cells and fibrosis. IgG4-related kidney disease in the renal pelvis is rare.

Case presentation

We describe a 53-year-old Asian woman who was referred to our hospital with a space-occupying renal lesion discovered by medical examination. A physical examination and laboratory evaluation revealed no significant abnormalities. Computed tomography scans showed a soft-tissue mass with an irregular border and mild homogeneous enhancement in the right renal pelvis and calyces. A positron emission tomography/computed tomography scan revealed soft-tissue density shadows with increased radionuclide uptake. To investigate a suspected pelvic carcinoma, a right ureteronephrectomy was performed. A pathologic examination of the renal sections showed a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, with fibrosis beneath the urothelial epithelium of the renal pelvis. Postoperatively, the serum IgG4 level was significantly elevated. The patient was diagnosed with IgG4-related kidney disease.


We present a case of IgG4-related kidney disease mimicking urothelial carcinoma in the renal pelvis. When a buried and solitary hypovascular tumor is detected in the kidney, we must consider IgG4-related kidney disease as a differential diagnosis. Accordingly, elevated serum IgG4, radiologic findings, and pathologic examination may improve the diagnosis.
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