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Title: Clinical features and outcomes in a cohort of patients with immunoglobulin G4-related disease at a university hospital in Spain
Author: Quero Ramos, Maria
Draibe, Juliana
Solanich, Xavier
Rama, Inés
Gomà, Montse
Martínez Valenzuela, Laura
Fulladosa, Xavier
Cruzado, Josep Ma.
Torras, Joan
Keywords: Glucocorticoides
Immunoglobulina G
Hospitals universitaris
Immunoglobulin G
University hospitals
Issue Date: 2019
Publisher: Oxford University Press
Abstract: Background. Immunoglobulin G4-related disease (IgG4-RD) is a fibro-inflammatory, immune-mediated disorder, which characteristically affects the glandular tissue but has the potential to affect any organ. Methods. We retrospectively reviewed clinical, laboratory, histological characteristics and treatment response during 12 months of follow-up of a cohort of patients with IgG4-RD diagnosed at a tertiary public hospital. Disease activity was assessed by means of the IgG4-RD responder index (IgG4-RD RI). Results. In all, 15 patients have been diagnosed at our Institution and herein studied (80% men), with a median age of 60.7 years and a mean affectation of 2.8 organs per patient. We identified six patients with definitive diagnosis and nine with possible IgG4-RD, according to the Japanese diagnostic algorithm. IgG4-RD RI decreased from a median of 11.3 at baseline to 4.0 after 6 months and 6.2 after 12 months. Relapse occurred in five patients and was associated with lower cumulative steroid doses. Five patients (33.3%) required additional immunosuppressive (IS) drugs. Five adverse events were seen during follow-up: three infections, one deep vein thrombosis and one gastrointestinal bleeding. One patient died of pneumonia. Conclusions. IgG4-RD is an inflammatory disease that can affect any organ. Glucocorticoids were an effective first line of treatment; however, this treatment is associated with important adverse events and relapses occurred in patients with low cumulative doses. As an alternative, IS treatment with rituximab could be an interesting option in those patients.
Note: Reproducció del document publicat a:
It is part of: Clinical Kidney Journal, 2019, vol. 12, num. 6, p. 829-835
Related resource:
ISSN: 2048-8505
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Ciències Clíniques)

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