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RMD Open. 2019 Jan 19;4(Suppl 1):e000787. doi: 10.1136/rmdopen-2018-000787. eCollection 2018.

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

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Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy.
Rheumatology Unit, AOU Pisana, Pisa, Italy.
Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
Department of Internal Medicine, Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
Department of Rheumatology and Clinical Immunology, Charité-University Medicine Berlin, Berlin, Germany.
Department of Internal Medicine and Immunology, Erasmus MC, Rotterdam, The Netherlands.
7, Koersel, Belgium.
Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France.
Division of Rheumatology, University and IRCCS Policlinico S Matteo Foundation, Pavia, Italy.
Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy.
Clinical Immunology and Rheumatology, Amsterdam Rheumatology and Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands.
University of Pisa, Pisa, Italy.
Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Bad Nauheim, Germany.
Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
Department of Internal Medicine, Ghent University, Ghent, Belgium.
Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.
Rheumatology Unit, University of Pisa, Pisa, Italy.


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.


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

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