title: The Clinical and Pathological Features of IgG(4)-Related Disease.
abstract: The rapidly emerging disorder now known as IgG(4)-related disease (IgG(4)-RD) includes a variety of clinical entities once regarded as being entirely separate diseases. Manifestations of IgG(4)-RD have now been reported in essentially all organ systems. Regardless of which organ is involved, tissue biopsies reveal striking histopathological similarities. The hallmark pathology findings are diffuse lymphoplasmacytic infiltrates, abundant IgG(4)-positive plasma cells, modest tissue eosinophilia, and extensive fibrosis. Tumorous swelling and obliterative phlebitis are other frequently observed features. Polyclonal elevations of serum IgG(4) are found in approximately 70% of patients. Many questions pertaining to the etiology, pathophysiology, epidemiology, clinical features, therapy, disease monitoring, and long-term outcomes remain to be addressed. This paper focuses on the clinical and pathological features of IgG(4)-RD.
authors.affiliation: Rheumatology Unit (Department of Medicine), Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
authors.forename: Arezou
authors.initials: A
authors.lastname: Khosroshahi
authors.forename: Vikram
authors.initials: V
authors.lastname: Deshpande
authors.forename: John H
authors.initials: JH
authors.lastname: Stone
mesh_terms: D004802:Eosinophilia / Q000276:immunology / Q000473:pathology; D005355:Fibrosis / Q000276:immunology / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008214:Lymphocytes / Q000276:immunology / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000276:immunology* / Q000473:pathology* / Q000503:physiopathology; D010689:Phlebitis / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology
chemical_list: D007074:Immunoglobulin G