RT Journal Article
T1 IgG4-Related Disease: Results From a Multicenter Spanish Registry.
A1 Fernández-Codina, Andreu
A1 Martínez-Valle, Fernando
A1 Pinilla, Blanca
A1 López, Cristina
A1 DeTorres, Inés
A1 Solans-Laqué, Roser
A1 Fraile-Rodríguez, Guadalupe
A1 Casanovas-Martínez, Arnau
A1 López-Dupla, Miguel
A1 Robles-Marhuenda, Ángel
A1 Barragán-González, María Jesús
A1 Cid, Maria Cinta
A1 Prieto-González, Sergio
A1 Brito-Zerón, Pilar
A1 Cruces-Moreno, María Teresa
A1 Fonseca-Aizpuru, Eva
A1 López-Torres, Manuel
A1 Gil, Judith
A1 Núñez-Fernández, Manuel Jesús
A1 Pardos-Gea, José
A1 Salvador-Cervelló, Gonzalo
K1 Humanos
K1 Enfermedades autoinmunes
K1 Inmunosupresores
K1 Inmunoglobulina G
K1 Paraproteinemias
K1 Sistema de registros
K1 España
AB 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.
PB Lippincott, Williams & Wilkins
SN 0025-7974
YR 2015
FD 2015-08-14
LK http://hdl.handle.net/10668/2552
UL http://hdl.handle.net/10668/2552
LA en
NO 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
NO Journal Article; Multicenter Study; Observational Study;
DS RISalud
RD Apr 11, 2025