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Case Report
Korean J Med. 2014;87(6):710-715. Published online December 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.87.6.710
신우 종괴 형성과 췌장 침범을 동반한 IgG4 연관 경화성 질환 1예
김우재1, 노명환1, 류충헌1, 한승희1, 남화성1, 한지은1, 홍숙희2
1동아대학교 의과대학 내과학교실
2동아대학교 의과대학 병리학교실
A Case of IgG4-Related Pancreas and Kidney Disease Mimicking a Renal Pelvic Malignancy
Woo Jae Kim1, Myung Hwan Noh1, Choong Heon Ryu1, Seung Hee Han1, Hwa Seong Nam1, Ji Eun Han1, Sook Hee Hong2
1Departments of Internal Medicine, Dong-A University College of Medicine, Busan, Korea
2Departments of Pathology, Dong-A University College of Medicine, Busan, Korea
Corresponding author: Myung Hwan Noh ,Tel: +82-51-240-2862, Fax: +82-51-242-5852, Email: mhnho@dau.ac.kr
Received: June 25, 2014; Revised: July 23, 2014   Accepted: July 31, 2014.


Abstract
IgG4-related sclerosing disease is a disease entity that has recently attracted attention, manifesting as a multiorgan disease characterized by high serum IgG4 levels, extensive IgG4-positive plasma cells and lymphocyte infiltration of the affected organs, with the pancreas (autoimmune pancreatitis) and kidney as representative targets. In cases of renal involvement, parenchymal lesions are predominant, such as renal cortical lesions or diffuse renal enlargement. However, mass-like lesions involving the renal pelvis are very rare, and mass forming or pelvic involvement types should be distinguished from lymphomas, metastatic cancers and other genitourinary malignancies to avoid unnecessary surgery. Herein, we report a case of IgG4-related sclerosing disease involving the kidney as an unusual involvement pattern presenting as a mass-like lesion with pelvic and perirenal involvement.

Keywords :IgG4-related kidney disease, Autoimmune pancreatitis, Renal pelvic mass
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