IgG4-related diseases: state of the art on clinical practice guidelines

• Published in: Rheumatic & musculoskeletal diseases open Vol. 4; no. Suppl 1; p. e000787
• Main Authors: Iaccarino, Luca, Talarico, Rosaria, Scirè, Carlo Alberto, Amoura, Zahir, Burmester, Gerd, Doria, Andrea, Faiz, Karim, Frank, Charissa, Hachulla, Eric, Hie, Miguel, Launay, David, Montecucco, Carlomaurizio, Monti, Sara, Mouthon, Luc, Tincani, Angela, Toniati, Paola, Van Hagen, Pieter Martin, Van Vollenhoven, Ronald F, Bombardieri, Stefano, Mueller-Ladner, Ulf, Schneider, Matthias, Smith, Vanessa, Cutolo, Maurizio, Mosca, Marta, Alexander, Tobias
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01.01.2019; BMJ; BMJ Publishing Group
• Series: RMD Open : Rheumatic & Musculoskeletal Diseases
• Subjects: Arthritis; Clinical medicine; Clinical practice guidelines; Connective Tissue Diseases; Immunoglobulins; Life Sciences; Patients; Systematic review; ERN reconnet; clinical practice guidelines; unmet needs; IgG4-related diseases; european reference networks
• ISSN: 2056-5933; 2056-5933
• DOI: 10.1136/rmdopen-2018-000787

Immunoglobulin G4-related diseases (IgG4-RD) are a group of chronic relapsing–remitting inflammatory conditions, characterised by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, fibrosis and a usually favourable response to steroids.In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the European Reference Network ReCONNET, aimed at evaluating existing clinical practice guidelines (CPGs) and recommendations in IgG4-RD. From 167 publications initially obtained from a systematic literature search, only one was identified as a systematic multispecialist, evidence-based, consensus guidance statement on diagnosis and treatment of IgG4-RD, which may be recommended for use as CPG in IgG4-RD.With the recognition of a limited evidence based in this increasingly recognised disease, the group discussion has identified the following unmet needs: lack of shared classification criteria, absence of formal guidelines on diagnosis, no evidence-based therapeutic recommendations and lack of activity and damage indices. Areas of unmet needs include the difficulties in diagnosis, management and monitoring and the scarcity of expert centres.