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Tytuł:
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Clinical phenotypes of IgG4-related disease reflect different prognostic outcomes.
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Autorzy:
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Lanzillotta M; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute.
Campochiaro C; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute.
Mancuso G; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute.
Ramirez GA; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute.
Capurso G; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Pancreato-Biliary Endoscopy and Endosonography Division, IRCCS San Raffaele Scientific Institute.
Falconi M; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Division of Pancreatic Surgery, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Dagna L; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute.
Della-Torre E; Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute.
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Źródło:
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Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2020 Sep 01; Vol. 59 (9), pp. 2435-2442.
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Typ publikacji:
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Journal Article; Observational Study
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Język:
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English
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Imprint Name(s):
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Original Publication: Oxford, UK : Avenel, N.J. : Oxford University Press ; Distributed by Mercury International, c1999-
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MeSH Terms:
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Latent Class Analysis*
Immunoglobulin G4-Related Disease/*classification
Aged ; Eosinophils/metabolism ; Female ; Glucocorticoids/therapeutic use ; Humans ; Immunoglobulin E/blood ; Immunoglobulin G/blood ; Immunoglobulin G4-Related Disease/diagnosis ; Immunologic Factors/therapeutic use ; Male ; Middle Aged ; Phenotype ; Predictive Value of Tests ; Prognosis ; Recurrence ; Reproducibility of Results ; Retrospective Studies ; Rituximab/therapeutic use
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Contributed Indexing:
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Keywords: IgG4; IgG4-related disease; aorta; pancreatitis; phenotypes; prognosis; retroperitoneal fibrosis; rituximab; treatment
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Substance Nomenclature:
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0 (Glucocorticoids)
0 (Immunoglobulin G)
0 (Immunologic Factors)
37341-29-0 (Immunoglobulin E)
4F4X42SYQ6 (Rituximab)
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Entry Date(s):
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Date Created: 20200628 Date Completed: 20210119 Latest Revision: 20210119
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Update Code:
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20240105
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DOI:
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10.1093/rheumatology/keaa221
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PMID:
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32591828
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Introduction: Four clinical phenotypes of IgG4-related disease (IgG4-RD) have been recently identified by latent class analysis (LCA): pancreato-biliary (group 1); retroperitoneum/aortitis (group 2); head and neck limited (group 3); and Mikulicz/systemic (group 4). The reproducibility of this classification in clinical practice and its relevance for patient management, however, remain unknown.
Methods: The study included 179 patients. Four IgG4-RD experts were asked to classify a validation cohort of 40 patients according to published LCA-derived phenotypes based on clinical judgement. Agreement between LCA and clinical clustering was calculated. To assess differences among disease phenotypes, the following variables were recorded on an additional 139 patients: serum IgG4 and IgE; inflammatory markers; eosinophils; plasmablasts; IgG4-RD responder index (RI); history of atopy, diabetes, osteoporosis, relapses and malignancy; cumulative dose of glucocorticoids; and use of rituximab.
Results: Clinical judgement replicated LCA classification with strong agreement among IgG4-RD experts (κ = 0.841, P < 0.0005). At disease onset, group 1 showed the highest levels of serum IgG4 and IgE. Groups 2 and 4 had the lowest and highest IgG4-RD RI, respectively. At 2 years' follow-up, group 3 received the highest cumulative dose of glucocorticoids, but higher incidences of diabetes mellitus were observed in groups 1 and 4, consistent with the higher likelihood of pancreatic involvement in groups 1 and 4. No difference among the four groups was observed in terms of disease recurrence, time to relapse and frequency of rituximab infusion.
Conclusion: Clinical phenotypes of IgG4-RD reflect differences in epidemiological features and prognostic outcomes.
(© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
Comment in: Rheumatology (Oxford). 2020 Sep 1;59(9):2195-2196. (PMID: 32594181)
