Author, as appears in the article.: Fernández-Codina, A; Martínez-Valle, F; Pinilla, B; López, C; DeTorres, I; Solans-Laqué, R; Fraile-Rodríguez, G; Casanovas-Martínez, A; López-Dupla, M; Robles-Marhuenda, A; Barragán-González, MJ; Cid, MC; Prieto-González, S; Brito-Zerón, P; Cruces-Moreno, MT; Fonseca-Aizpuru, E; López-Torres, M; Gil, J; Núñez-Fernández, MJ; Pardos-Gea, J; Salvador-Cervelló, G
Department: Medicina i Cirurgia
URV's Author/s: López Dupla, Jesús Miguel
Keywords: Systemic-disease Retroperitoneal fibrosis Prednisone Cohort Autoimmune pancreatitis
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.
Thematic Areas: Saúde coletiva Odontología Nutrição Medicine, general & internal Medicine (miscellaneous) Medicine (all) Medicina iii Medicina ii Medicina i Matemática / probabilidade e estatística Interdisciplinar Geografía General medicine Farmacia Ensino Engenharias iv Enfermagem Educação física Ciências biológicas iii Ciências biológicas ii Ciências biológicas i Biotecnología Astronomia / física Antropologia / arqueologia
licence for use: https://creativecommons.org/licenses/by/3.0/es/
Author's mail: jesusmiguel.lopez@urv.cat
Author identifier: 0000-0002-9141-2523
Record's date: 2024-09-07
Papper version: info:eu-repo/semantics/publishedVersion
Link to the original source: https://journals.lww.com/md-journal/fulltext/2015/08020/igg4_related_disease__results_from_a_multicenter.21.aspx
Licence document URL: https://repositori.urv.cat/ca/proteccio-de-dades/
Papper original source: Medicine. 94 (32):
APA: Fernández-Codina, A; Martínez-Valle, F; Pinilla, B; López, C; DeTorres, I; Solans-Laqué, R; Fraile-Rodríguez, G; Casanovas-Martínez, A; López-Dupla, M (2015). IgG4-Related Disease: Results From a Multicenter Spanish Registry. Medicine, 94(32), -. DOI: 10.1097/MD.0000000000001275
Article's DOI: 10.1097/MD.0000000000001275
Entity: Universitat Rovira i Virgili
Journal publication year: 2015
Publication Type: Journal Publications