Immunoglobulin G4-Related Systemic Sclerosing Disease: A Case Involving the Ureter and Kidney

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 we...

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Detalles Bibliográficos
Autores principales: Kim, Sunchan, Kim, Tae Gu, Choi, Seung-Kwon, Kim, Myung Joon, Min, Gyeong Eun, Lee, Hyung-Lae, Yoo, Koo Han
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Urological Association 2013
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3604578/
https://www.ncbi.nlm.nih.gov/pubmed/23525099
http://dx.doi.org/10.4111/kju.2013.54.3.209
Descripción
Sumario: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.

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