title: [IgG4-related disease is a rare differential diagnosis of malignant and autoimmune diseases].
abstract: Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory and fibrotic disease with the potential to produce diffuse enlargement, massforming lesions or stenoses in a wide range of organs. Elevation of serum IgG4 concentration and high levels of IgG4-positive cells in the inflamed tissue are common denominators. Type 1 autoimmune pancreatitis is one of the main manifestations, and its recognition preceded the definition of IgG4-RD as a novel clinical entity. The aetiology, pathophysiology, epidemiology and clinical long-term outcome of IgG4-RD are not fully elucidated. Steroids are effective in most patients, sometimes combined with other antiinflammatory drugs.
authors: Afdeling for Klinisk Patologi, Odense Universitetshospital, Winsløwparken 15, 5000 Odense C. sonke.detlefsen@rsyd.dk.
authors: Anders
authors: A
authors: Storgaard
authors: Sönke
authors: S
authors: Detlefsen
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D003937:Diagnosis, Differential; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D010195:Pancreatitis / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G