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

ABSTRACT

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.