Reviews
Blood biomarkers recommended for diagnosing and monitoring IgG4-related disease. Considerations from the ERN ReCONNET and collaborating partners
L. Iaccarino1, R. Talarico2, E. Bozzalla-Cassione3, G.R. Burmester4, E.L. Culver5, A. Doria6, M. Ebbo7, P.M. Van Hagen8, E. Hachulla9, J.A. Van Laar10, M. Lanzillotta11, F. Martinez-Valle12, C. Montecucco13, S. Monti14, C. Nalli15, N. Schleinitz16, A. Tincani17, E. Della-Torre18, T. Alexander19
- Rheumatology Unit, Department of Medicine-DIMED, University Hospital of Padua, Italy.
- Rheumatology Unit, AOU Pisana, Pisa, Italy.
- Division of Rheumatology, IRCCS Policlinico S. Matteo Foundation and University of Pavia, Italy.
- Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and the Berlin Institute of Health (BIH), Berlin, Germany.
- Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK.
- Rheumatology Unit, Department of Medicine-DIMED, University Hospital of Padua, Italy.
- Department of Internal Medicine, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France.
- Departments of Internal Medicine and Immunology, Section Clinical Immunology, Erasmus MC, Rotterdam, the Netherlands.
- Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Université de Lille, CHU Lille, France.
- Departments of Internal Medicine and Immunology, Section Clinical Immunology, Erasmus MC, Rotterdam, the Netherlands.
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, and Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy.
- Systemic Autoimmune Diseases Unit, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institute of Research, Universitat Autònoma de Barcelona, Spain.
- Division of Rheumatology, IRCCS Policlinico S. Matteo Foundation and University of Pavia, Italy.
- Division of Rheumatology, IRCCS Policlinico S. Matteo Foundation and University of Pavia, Italy.
- Rheumatology and Immunology Unit, Spedali Civili and University of Brescia, ASST Spedali Civili of Brescia, Italy.
- Department of Internal Medicine, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France.
- Rheumatology and Immunology Unit, Spedali Civili and University of Brescia, ASST Spedali Civili of Brescia, Italy.
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, and Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy.
- Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and the Berlin Institute of Health (BIH), Berlin, and Deutsches Rheuma-Forschungszentrum (DRFZ Berlin) - a Leibniz Institute, Autoimmunology Group, Berlin, Germany. tobias.alexander@charite.de
CER15283
2022 Vol.40, N°5 ,Suppl.134
PI 0071, PF 0080
Reviews
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PMID: 35238758 [PubMed]
Received: 25/10/2021
Accepted : 10/01/2022
In Press: 01/03/2022
Published: 18/05/2022
Abstract
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic, clinically heterogenous fibroinflammatory condition, characterised by an accumulation of IgG4 secreting plasma cells in affected tissues and associated with increased serum IgG4 concentrations. Despite a growing recognition of the disease among clinicians from different specialties worldwide, its indolent nature, lack of a single diagnostic test and ability to mimic other malignant, infective and inflammatory conditions, makes the diagnosis challenging. As treatment options evolve, biomarkers correlating with disease activity, predicting prognosis and response to treatment are deemed required. A multidisciplinary panel of experts from the European Reference Network for Rare and Complex Connective tissue diseases (ERN ReCONNET) and affiliated international partners have performed a narrative literature search and reviewed the current evidence of biomarkers in IgG4-RD, including immunoglobulins, cytokines, chemokines and other soluble immune mediators, and cellular components of the immune system. The aim of this paper is to provide useful information for clinicians as to the utility of biomarkers for diagnosing and monitoring IgG4-RD in clinical routine and sets out recommendations for clinical decision making.
