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Korean J Urol. 2013 Mar;54(3):209-11. doi: 10.4111/kju.2013.54.3.209. Epub 2013 Mar 15.

Immunoglobulin g4-related systemic sclerosing disease: a case involving the ureter and kidney.

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  • 1Department of Urology, Kyung Hee University Hospital at Gangdong, Seoul, Korea.


Immunoglobulin (Ig) G4-related sclerosing disease is a newly defined clinicopathological entity characterized by lymphoplasmacytic infiltration of IgG4-positive plasma cells and varying degrees of fibrosis within affected tissues. Patients usually exhibit multisystem involvement and often respond well to steroid and immunosuppressive therapy. This report presents a case of IgG4-related sclerosing disease involving the ureter and kidney. We hope to bring IgG4-related sclerosing disease to the attention of urologists, because it is an uncommon disease that commonly responds to systemic corticosteroids.


Immunoglobulin G; Sclerosis; Ureter

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