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

Luca Iaccarino, Rosaria Talarico, Carlo Alberto Scirè, Zahir Amoura, Gerd Burmester, Andrea Doria, Karim Faiz, Charissa Frank, Eric Hachulla, Miguel Hie, David Launay, Carlomaurizio Montecucco, Sara Monti, Luc Mouthon, Angela Tincani, Paola Toniati, Pieter Martin Van Hagen, Ronald F. Van Vollenhoven, Stefano Bombardieri, Ulf Mueller-LadnerMatthias Schneider, Vanessa Smith, Maurizio Cutolo, Marta Mosca, Tobias Alexander

Research output: Contribution to journalReview article


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.

Original languageEnglish
Article numbere000787
JournalRMD Open
Publication statusPublished - Jan 1 2019


  • clinical practice guidelines
  • ERN reconnet
  • european reference networks
  • IgG4-related diseases
  • unmet needs

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

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    Iaccarino, L., Talarico, R., Scirè, C. A., Amoura, Z., Burmester, G., Doria, A., Faiz, K., Frank, C., Hachulla, E., Hie, M., Launay, D., Montecucco, C., Monti, S., Mouthon, L., Tincani, A., Toniati, P., Van Hagen, P. M., Van Vollenhoven, R. F., Bombardieri, S., ... Alexander, T. (2019). IgG4-related diseases: State of the art on clinical practice guidelines. RMD Open, 4, [e000787]. https://doi.org/10.1136/rmdopen-2018-000787