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IgG4‐related retroperitoneal fibrosis: the first reported case in a Chinese population
Author(s) -
LIN YeongJang,
CHEN PeiChih,
CHEN HungAn,
LI ChienFeng
Publication year - 2010
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/j.1756-185x.2010.01543.x
Subject(s) - medicine , retroperitoneal fibrosis , chinese population , fibrosis , dermatology , pathology , genetics , genotype , gene , biology
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.