IgG4-related disease: results from a multicenter Spanish registry
| Autor: | Manuel López-Torres, Guadalupe Fraile-Rodríguez, Gonzalo Salvador-Cervelló, Sergio Prieto-González, Miguel López-Dupla, María Jesús Barragán-González, Ángel Robles-Marhuenda, Manuel Jesús Núñez-Fernández, José Pardos-Gea, María Teresa Cruces-Moreno, Cristina López, Eva Fonseca-Aizpuru, Andreu Fernández-Codina, Fernando Martínez-Valle, Judith Gil, Arnau Casanovas-Martínez, Blanca Pinilla, Pilar Brito-Zerón, Inés DeTorres, Roser Solans-Laqué, Maria C. Cid |
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| Přispěvatelé: | Universitat de Barcelona, [Fernández-Codina,A, Martínez-Valle,F, Solans-Laqué,R, Pardos-Gea,J] Internal Medicine Department, Hospital Universitari Vall d’Hebron, Barcelona, Spain. [Pinilla,B, López,C] Hospital General Universitario Gregorio Marañón, Madrid, Spain. [DeTorres,I] Pathology Department, Hospital Universitari Vall d’Hebron, Barcelona, Spain. [Fraile-Rodríguez,G] Hospital Ramón y Caja, Madrid, Spain. [Casanovas-Martínez,A.] Consorci Sanitari Parc Taulí, Spain. [López-Dupla,M] Hospital Universitari Joan XXIII, Tarragona, Spain. [Robles-Marhuenda,A] Hospital La Paz, Madrid, Spain. [Barragán-González,MJ] Hospital Valle del Nalón, Langreo, Spain. [Cid,MC, Prieto-González,S, Brito-Zerón,P] Hospital Clínic i Provincial, Barcelona, Spain. [Cruces-Moreno,MT] Hospital Universitario San Cecilio, Granada, Spain. [Fonseca-Aizpuru,E] Hospital de Cabueñes, Gijón, Spain. [López-Torres,M] Hospital Universitario La Princesa, Madrid, Spain. [Gil,J] Hospital Universitario Río Hortega, Valladolid, Spain. [Núñez-Fernández,MJ] Complejo Hospitalario de Pontevedra, Pontevedra, Spain. [Salvador-Cervelló,G] Hospital de Manises, Manises, Spain. |
| Předmět: |
medicine.medical_specialty
Epidemiology España Immunology Paraproteinemias Observational Study Disease Malignancy Autoimmune Diseases Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings] Malalties immunològiques Fibrosis Internal medicine Enfermedades autoinmunes Diseases::Immune System Diseases::Autoimmune Diseases [Medical Subject Headings] parasitic diseases medicine Humans Registries Immunologia Espanya Epidemiologia Geographicals::Geographic Locations::Europe::Spain [Medical Subject Headings] Chemicals and Drugs::Amino Acids Peptides and Proteins::Proteins::Globulins::Serum Globulins::Immunoglobulins::Antibodies::Immunoglobulin Isotypes::Immunoglobulin G [Medical Subject Headings] business.industry Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Protein Disorders::Paraproteinemias [Medical Subject Headings] General Medicine medicine.disease Inmunosupresores Surgery Humanos medicine.anatomical_structure Multicenter study Spain Inmunoglobulina G Immunoglobulin G Immunologic diseases Abdomen IgG4-related disease Sistema de registros Analytical Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Registries [Medical Subject Headings] Presentation (obstetrics) business Pancreas Chemicals and Drugs::Chemical Actions and Uses::Pharmacologic Actions::Physiological Effects of Drugs::Immunologic Factors::Immunosuppressive Agents [Medical Subject Headings] Immunosuppressive Agents Research Article |
| Zdroj: | Recercat. Dipósit de la Recerca de Catalunya instname Dipòsit Digital de la UB Universidad de Barcelona Medicine Repositorio Institucional de la Consejería de Sanidad de la Comunidad de Madrid Consejería de Sanidad de la Comunidad de Madrid Europe PubMed Central |
| Popis: | Journal Article; Multicenter Study; Observational Study; IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients.To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients.A retrospective multicenter study was performed. Twelve hospitals across Spain included patients meeting the current 2012 consensus criteria on IgG4-RD diagnosis.Fifty-five patients were included in the study, 38 of whom (69.1%) were male. Median age at diagnosis was 53 years. Thirty (54.5%) patients were included in the Histologically Highly Suggestive IgG4-RD group and 25 (45.5%) in the probable IgG4-RD group. Twenty-six (47.3%) patients had more than 1 organ affected at presentation. The most frequently affected organs were: retroperitoneum, orbital pseudotumor, pancreas, salivary and lachrymal glands, and maxillary sinuses.Corticosteroids were the mainstay of treatment (46 patients, 83.6%). Eighteen patients (32.7%) required additional immunosuppressive agents. Twenty-four (43.6%) patients achieved a complete response and 26 (43.7%) presented a partial response ( |
| Databáze: | OpenAIRE |
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