Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework

Della-Torre E, Talarico R, Ballarin J, Bozzalla-Cassione E, Cardamone C, Cigolini C, Ferro F, Fonseca T, Fragoulis GE, Galetti I, Gerosa M, Hernández-Rodríguez J, Lanzillotta M, Marinello D, Martin T, Martinez-Valle F, Maślińska M, Moretti M, Mosca M, Müller-Ladner U, Nalli C, Orsolini G, Pamfil C, Perez-Garcia G, Priori R, Quattrocchio G, Ramming A, Regola F, Romão VC, Silva A, van Laar JA, Vicente-Edo MJ, Vinker S, Alexander T

Publication Type: Journal article, Review article

Journal

DOI: 10.1016/S2665-9913(24)00192-9

Abstract

IgG4-related disease is a rare fibroinflammatory condition. Prompt recognition is fundamental to initiate treatment and to prevent organ damage. Diagnostic and classification criteria are primarily intended for use by clinicians with established expertise in IgG4-related disease. Absence of disease awareness among primary care physicians and specialists without expertise in IgG4-related disease remains the main cause of diagnostic delay. We aimed to identify red flags that might increase the suspicion of IgG4-related disease in primary and secondary care settings. A task force of experts in IgG4-related disease from the European Reference Network for Rare Connective Tissue Diseases (ERN-ReCONNET), patient representatives, and primary care physicians derived potential red flags for IgG4-related disease through a systematic literature search and a level of agreement exercise. Five red flags reached 100% agreement among experts: swelling in one or more organ system; pancreatic and biliary tree involvement; increased serum IgG4; IgG4⁺ plasma cell tissue infiltration; and obliterative phlebitis. Red flags for IgG4-related disease are intended for use in primary and secondary care to improve referral to centres of expertise and prompt early diagnosis.

Authors with CRIS profile

Involved external institutions

Instituto Aragonés de Ciencias de la Salud (IACS) Spain (ES) Azienda Ospedaliero-Universitaria Pisana Italy (IT) Berliner Institut für Gesundheitsforschung (BIH) Germany (DE) National and Kapodistrian University of Athens Greece (GR) Justus-Liebig-Universität Gießen Germany (DE) Federation of European Scleroderma Associations (FESCA) Belgium (BE) Università degli Studi di Brescia Italy (IT) University Hospital of Verona / Azienda Ospedaliera Universitaria Integrata Verona (AOUI) Italy (IT) Università degli studi di Milano Italy (IT) Iuliu Hațieganu University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie "Iuliu Hațieganu" (UMF Cluj) Romania (RO) Universitat de Barcelona (UB) / University of Barcelona Spain (ES) Università Vita-Salute San Raffaele (UniSR) Italy (IT) Policlinico Umberto I Italy (IT) University of Turin / Università degli Studi di Torino (UNITO) Italy (IT) Hôpitaux universitaires de Strasbourg (HUS) / University Hospital Strasbourg France (FR) Vall d'Hebron University Hospital / Hospital Universitari Vall d'Hebron Spain (ES) University of Lisbon / Universidade de Lisboa (ULisboa) Portugal (PT) Erasmus University Medical Center (MC) Netherlands (NL) National Institute of Geriatrics, Rheumatology and Rehabilitation / Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji Poland (PL) European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET) Italy (IT) BIOMEdical Research Informatics Solutions Italy (IT) Ospedali Riuniti San Giovanni di Dio e Ruggi d'Aragona Italy (IT)

How to cite

APA:

Della-Torre, E., Talarico, R., Ballarin, J., Bozzalla-Cassione, E., Cardamone, C., Cigolini, C.,... Alexander, T. (2024). Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework. The Lancet Rheumatology. https://doi.org/10.1016/S2665-9913(24)00192-9

MLA:

Della-Torre, Emanuel, et al. "Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework." The Lancet Rheumatology (2024).

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