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Title: IgG4-Related Disease: Results From a Multicenter Spanish Registry.
Authors: Fernández-Codina, Andreu
Martínez-Valle, Fernando
Pinilla, Blanca
López, Cristina
DeTorres, Inés
Solans-Laqué, Roser
Fraile-Rodríguez, Guadalupe
Casanovas-Martínez, Arnau
López-Dupla, Miguel
Robles-Marhuenda, Ángel
Barragán-González, María Jesús
Cid, Maria Cinta
Prieto-González, Sergio
Brito-Zerón, Pilar
Cruces-Moreno, María Teresa
Fonseca-Aizpuru, Eva
López-Torres, Manuel
Gil, Judith
Núñez-Fernández, Manuel Jesús
Pardos-Gea, José
Salvador-Cervelló, Gonzalo
metadata.dc.contributor.authoraffiliation: [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.
Keywords: Humanos;Enfermedades autoinmunes;Inmunosupresores;Inmunoglobulina G;Paraproteinemias;Sistema de registros;España
metadata.dc.subject.mesh: Medical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Globulins::Serum Globulins::Immunoglobulins::Antibodies::Immunoglobulin Isotypes::Immunoglobulin G
Medical Subject Headings::Chemicals and Drugs::Chemical Actions and Uses::Pharmacologic Actions::Physiological Effects of Drugs::Immunologic Factors::Immunosuppressive Agents
Medical Subject Headings::Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Protein Disorders::Paraproteinemias
Medical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Registries
Medical Subject Headings::Diseases::Immune System Diseases::Autoimmune Diseases
Medical Subject Headings::Geographicals::Geographic Locations::Europe::Spain
Issue Date: 14-Aug-2015
Publisher: Lippincott, Williams & Wilkins
Citation: Fernández-Codina A, Martínez-Valle F, Pinilla B, López C, DeTorres I, Solans-Laqué R, et al. IgG4-Related Disease: Results From a Multicenter Spanish Registry. Medicine (Baltimore). 2015 ; 94(32):e1275
Abstract: 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 (<50% of regression) after 22 months of follow-up. No deaths were attributed directly to IgG4-RD and malignancy was infrequent.This is the largest IgG4-RD series reported in Europe. Patients were middle-aged males, with histologically probable IgG4-RD. The systemic form of the disease was frequent, involving mainly sites of the head and abdomen. Corticosteroids were an effective first line treatment, sometimes combined with immunosuppressive agents. Neither fatalities nor malignancies were attributed to IgG4-RD.
Description: Journal Article; Multicenter Study; Observational Study;
metadata.dc.identifier.doi: 10.1097/MD.0000000000001275
ISSN: 1536-5964 (Online)
0025-7974 (Print)
Appears in Collections:01- Artículos - Complejo Hospitalario Universitario de Granada

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