title: IgG4-related Disease from Head to Toe.
abstract: Immunoglobulin G4 (IgG4)-related disease is a relatively recently proposed clinical-pathologic entity that is characterized by fibro-inflammatory lesions rich in IgG4-positive plasma cells and, often but not always, elevated serum IgG4 concentrations. IgG4-related disease was recognized as a systemic disease in 2003, when extrapancreatic manifestations were identified in patients with autoimmune pancreatitis. Since then, the disease has been reported as affecting virtually every organ system and has been identified in the biliary tree, salivary and lacrimal glands, periorbital tissues, lungs, lymph nodes, thyroid gland, kidneys, prostate gland, testicles, breasts, and pituitary gland. Its pathogenesis is poorly understood, but findings are consistent with both an autoimmune and an allergic disorder. Although definitive diagnosis requires histopathologic analysis, imaging plays an important role in demonstrating infiltration and enlargement of involved organs. Because of the systemic nature of the disease, imaging workup of IgG4-related disease should always include whole-body examinations to detect multiorgan involvement. Patients often present with subacute development of a mass in or diffuse enlargement of the affected organ, sometimes mimicking a neoplastic process. In every anatomic location, several inflammatory and neoplastic entities must be considered in the differential diagnosis. Because IgG4-related disease usually shows a marked response to corticosteroid therapy, radiologists should be familiar with its clinical and imaging manifestations to avoid a delay in diagnosis and unnecessary surgical interventions.
authors.affiliation: From the Departments of Radiology (A.M.d.A., S.B.G., R.G.F., A.B.N., C.V.M.), Pathology (I.A.N.), and Internal Medicine (J.A.D.P.), Hospital Clínico Universitario de Santiago de Compostela, Choupana s/n, 15706 Santiago de Compostela (A Coruña), Spain.
authors.forename: Anxo
authors.initials: A
authors.lastname: Martínez-de-Alegría
authors.affiliation: From the Departments of Radiology (A.M.d.A., S.B.G., R.G.F., A.B.N., C.V.M.), Pathology (I.A.N.), and Internal Medicine (J.A.D.P.), Hospital Clínico Universitario de Santiago de Compostela, Choupana s/n, 15706 Santiago de Compostela (A Coruña), Spain.
authors.forename: Sandra
authors.initials: S
authors.lastname: Baleato-González
authors.affiliation: From the Departments of Radiology (A.M.d.A., S.B.G., R.G.F., A.B.N., C.V.M.), Pathology (I.A.N.), and Internal Medicine (J.A.D.P.), Hospital Clínico Universitario de Santiago de Compostela, Choupana s/n, 15706 Santiago de Compostela (A Coruña), Spain.
authors.forename: Roberto
authors.initials: R
authors.lastname: García-Figueiras
authors.affiliation: From the Departments of Radiology (A.M.d.A., S.B.G., R.G.F., A.B.N., C.V.M.), Pathology (I.A.N.), and Internal Medicine (J.A.D.P.), Hospital Clínico Universitario de Santiago de Compostela, Choupana s/n, 15706 Santiago de Compostela (A Coruña), Spain.
authors.forename: Anaberta
authors.initials: A
authors.lastname: Bermúdez-Naveira
authors.affiliation: From the Departments of Radiology (A.M.d.A., S.B.G., R.G.F., A.B.N., C.V.M.), Pathology (I.A.N.), and Internal Medicine (J.A.D.P.), Hospital Clínico Universitario de Santiago de Compostela, Choupana s/n, 15706 Santiago de Compostela (A Coruña), Spain.
authors.forename: Ihab
authors.initials: I
authors.lastname: Abdulkader-Nallib
authors.affiliation: From the Departments of Radiology (A.M.d.A., S.B.G., R.G.F., A.B.N., C.V.M.), Pathology (I.A.N.), and Internal Medicine (J.A.D.P.), Hospital Clínico Universitario de Santiago de Compostela, Choupana s/n, 15706 Santiago de Compostela (A Coruña), Spain.
authors.forename: José A
authors.initials: JA
authors.lastname: Díaz-Peromingo
authors.affiliation: From the Departments of Radiology (A.M.d.A., S.B.G., R.G.F., A.B.N., C.V.M.), Pathology (I.A.N.), and Internal Medicine (J.A.D.P.), Hospital Clínico Universitario de Santiago de Compostela, Choupana s/n, 15706 Santiago de Compostela (A Coruña), Spain.
authors.forename: Carmen
authors.initials: C
authors.lastname: Villalba-Martín
mesh_terms: D001327:Autoimmune Diseases / Q000000981:diagnostic imaging*; D015209:Cholangitis, Sclerosing / Q000000981:diagnostic imaging* / Q000276:immunology / Q000473:pathology; D006801:Humans; D006942:Hypergammaglobulinemia / Q000000981:diagnostic imaging*; D007074:Immunoglobulin G; D007765:Lacrimal Apparatus / Q000000981:diagnostic imaging; D008220:Lymphography; D008643:Mesentery / Q000000981:diagnostic imaging; D009915:Orbit / Q000000981:diagnostic imaging; D010179:Pancreas / Q000000981:diagnostic imaging; D050500:Pancreatitis, Chronic / Q000000981:diagnostic imaging* / Q000276:immunology / Q000473:pathology; D012137:Respiratory System / Q000000981:diagnostic imaging; D012796:Sialography; D012859:Sjogren's Syndrome / Q000000981:diagnostic imaging* / Q000276:immunology / Q000473:pathology; D013961:Thyroid Gland / Q000000981:diagnostic imaging; D013967:Thyroiditis, Autoimmune / Q000000981:diagnostic imaging
chemical_list: D007074:Immunoglobulin G