Blood biomarkers recommended for diagnosing and monitoring IgG4-related disease. Considerations from the ERN ReCONNET and collaborating partners

Luca Iaccarino, Rosaria Talarico, Emanuele Bozzalla-Cassione, Gerd R. Burmester, Emma L. Culver, Andrea Doria, Mikael Ebbo, P. Martin van Hagen, Eric Hachulla, Jan A.M. van Laar, Marco Lanzillotta, Fernando Martinez-Valle, Carlomaurizio Montecucco, Sara Monti, Cecilia Nalli, Nicolas Schleinitz, Angela Tincani, Emanuel Della-Torre, Tobias Alexander*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

10 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)S71-S80
JournalClinical and Experimental Rheumatology
Volume40
Issue number5
DOIs
Publication statusPublished - May 2022

Bibliographical note

This publication was funded by the European Union’s Health Programme (2014-2020).
ERN ReCONNET is one of the 24 European Reference Networks (ERNs) approved by the ERN Board of Member
States. The ERNs are co-funded by the EC (European Commission). The content of this publication represents
the views of the authors only and it is their sole responsibility; it cannot be considered to reflect the views of the
EC and/or the European Health and Digital Executive Agency (HaDEA) or any other body of the European Union.
The EC and HaDEA do not accept any responsibility for use that may be made of the information it contains.

Publisher Copyright: © 2022 Clinical and Experimental Rheumatology S.A.S.. All rights reserved.

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