IgG4-related retroperitoneal fibrosis: the first reported case in a Chinese population

Immunoglobulin G4 (IgG4)-related sclerosing disease is a newly recognized clinicopathological entity characterized by lymphoplasmacytic infiltration and varying degrees of fibrosis in various organs, with abundant IgG4-positive plasma cells in tissues. Patients usually exhibit multisystem involvement and often respond well to steroid and immunosuppressive therapy. However, this disease has been rarely reported in a Chinese population. We herein report a case of IgG4-related sclerosing disease solely presenting with retroperitoneal fibrosis that was effectively treated with systemic steroid therapy. To the best of our knowledge, this is the first reported case of IgG4-related retroperitoneal fibrosis in a Chinese population.