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Title: IgG4-related disease: results from a multicenter Spanish registry
Author: 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 Xutglà, M. Cinta
Prieto González, Sergio
Brito Zerón, María del Pilar
Cruces Moreno, María Teresa
Fonseca Aizpuru, Eva
López Torres, Manuel
Gil, Judith
Núñez Fernández, Manuel Jesús
Pardos Gea, José
Salvador Cervelló, Gonzalo
Keywords: Malalties immunològiques
Immunologic diseases
Issue Date: Aug-2015
Publisher: Lippincott, Williams & Wilkins. Wolters Kluwer Health
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.
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It is part of: Medicine, 2015, vol. 94, num. 32, p. e1275-e1275
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ISSN: 0025-7974
Appears in Collections:Articles publicats en revistes (Medicina)

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