Emanuel Della-Torre, Rosaria Talarico, Jose Ballarin, Emanuele Bozzalla-Cassione, Chiara Cardamone, Cosimo Cigolini, Francesco Ferro, Tomas Fonseca, George E Fragoulis, Ilaria Galetti, Maria Gerosa, José Hernández-Rodríguez, Marco Lanzillotta, Diana Marinello, Thierry Martin, Fernando Martinez-Valle, Maria Maślińska, Michele Moretti, Marta Mosca, Ulf Müller-Ladner, Cecilia Nalli, Giovanni Orsolini, Cristina Pamfil, Guillermo Perez-Garcia, Roberta Priori, Giacomo Quattrocchio, Andreas Ramming, Francesca Regola, Vasco C Romão, Augusto Silva, Jan A M van Laar, Maria Jose Vicente-Edo, Shlomo Vinker, Tobias Alexander
{"title":"Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework.","authors":"Emanuel Della-Torre, Rosaria Talarico, Jose Ballarin, Emanuele Bozzalla-Cassione, Chiara Cardamone, Cosimo Cigolini, Francesco Ferro, Tomas Fonseca, George E Fragoulis, Ilaria Galetti, Maria Gerosa, José Hernández-Rodríguez, Marco Lanzillotta, Diana Marinello, Thierry Martin, Fernando Martinez-Valle, Maria Maślińska, Michele Moretti, Marta Mosca, Ulf Müller-Ladner, Cecilia Nalli, Giovanni Orsolini, Cristina Pamfil, Guillermo Perez-Garcia, Roberta Priori, Giacomo Quattrocchio, Andreas Ramming, Francesca Regola, Vasco C Romão, Augusto Silva, Jan A M van Laar, Maria Jose Vicente-Edo, Shlomo Vinker, Tobias Alexander","doi":"10.1016/S2665-9913(24)00192-9","DOIUrl":null,"url":null,"abstract":"<p><p>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<sup>+</sup> 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.</p>","PeriodicalId":48540,"journal":{"name":"Lancet Rheumatology","volume":null,"pages":null},"PeriodicalIF":15.0000,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Lancet Rheumatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/S2665-9913(24)00192-9","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0
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.
期刊介绍:
The Lancet Rheumatology, an independent journal, is dedicated to publishing content relevant to rheumatology specialists worldwide. It focuses on studies that advance clinical practice, challenge existing norms, and advocate for changes in health policy. The journal covers clinical research, particularly clinical trials, expert reviews, and thought-provoking commentary on the diagnosis, classification, management, and prevention of rheumatic diseases, including arthritis, musculoskeletal disorders, connective tissue diseases, and immune system disorders. Additionally, it publishes high-quality translational studies supported by robust clinical data, prioritizing those that identify potential new therapeutic targets, advance precision medicine efforts, or directly contribute to future clinical trials.
With its strong clinical orientation, The Lancet Rheumatology serves as an independent voice for the rheumatology community, advocating strongly for the enhancement of patients' lives affected by rheumatic diseases worldwide.