Immunglobulin G4-relateret sygdom er en sjaelden differentialdiagnose til maligne og autoimmune sygdomme
Engelsk titel: IgG4-related disease is a rare differential diagnosis of malignant and autoimmune diseases
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Författare:
Detlefsen, Sönke
;
Storgaard, Anders
Email: sonke.detlefsen@rsyd.dk
Språk: Dan
Antal referenser: 30
Dokumenttyp:
Översikt
UI-nummer: 16047222
Sammanfattning
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 anti-inflammatory drugs.