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

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.

• Authors: 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
• Journal: Clinical and experimental rheumatology (Clin Exp Rheumatol) Vol. 40 Suppl 134 Issue 5 Pg. 71-80 (May 2022) ISSN: 0392-856X [Print] Italy
• PMID: 35238758 (Publication Type: Journal Article, Review)

Chemical References

• Biomarkers
• Chemokines
• Immunoglobulin G

Topics

• Autoimmune Diseases (diagnosis)
• Biomarkers
• Chemokines
• Humans
• Immunoglobulin G
• Immunoglobulin G4-Related Disease (diagnosis)
• Plasma Cells (pathology)