IgG4-Related Disease: Results From a Multicenter Spanish Registry.
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.
From the Internal Medicine Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain (AF-C, FM-V, RS-L, JP-G); Hospital General Universitario Gregorio Marañón, Madrid, Spain (BP, CL); Pathology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain (IDT); Hospital Ramón y Caja, Madrid, Spain (GF-R); Consorci Sanitari Parc Taulí, Spain (AC-M); Hospital Universitari Joan XXIII, Tarragona, Spain (ML-D); Hospital La Paz, Madrid, Spain (AR-M); Hospital Valle del Nalón, Langreo, Spain (MJB-G); Hospital Clínic i Provincial, Barcelona, Spain (MCC, SP-G, PB-Z); Hospital Universitario San Cecilio, Granada, Spain (MTC-M); Hospital de Cabueñes, Gijón, Spain (EF-A); Hospital Universitario La Princesa, Madrid, Spain (ML-T); Hospital Universitario Río Hortega, Valladolid, Spain (JG); Complejo Hospitalario de Pontevedra, Pontevedra, Spain (MJN-F); and Hospital de Manises, Manises, Spain (GS-C).
Andreu
A
Fernández-Codina
Fernando
F
Martínez-Valle
Blanca
B
Pinilla
Cristina
C
López
Inés
I
DeTorres
Roser
R
Solans-Laqué
Guadalupe
G
Fraile-Rodríguez
Arnau
A
Casanovas-Martínez
Miguel
M
López-Dupla
Ángel
Á
Robles-Marhuenda
María Jesús
MJ
Barragán-González
Maria Cinta
MC
Cid
Sergio
S
Prieto-González
Pilar
P
Brito-Zerón
María Teresa
MT
Cruces-Moreno
Eva
E
Fonseca-Aizpuru
Manuel
M
López-Torres
Judith
J
Gil
Manuel Jesús
MJ
Núñez-Fernández
José
J
Pardos-Gea
Gonzalo
G
Salvador-Cervelló
D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D006801:Humans; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010265:Paraproteinemias / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology; D012042:Registries; D013030:Spain / Q000453:epidemiology
D007074:Immunoglobulin G; D007166:Immunosuppressive Agents