{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T20:53:37Z","timestamp":1789073617393,"version":"build-2803163510"},"reference-count":20,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2024,11,17]],"date-time":"2024-11-17T00:00:00Z","timestamp":1731801600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Sociedad Espa\u00f1ola de Reumatolog\u00eda"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Introduction: We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College of Rheumatology\/European League Against Rheumatism (ACR\/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD). Materials and Methods: A retrospective study was conducted in a single tertiary hospital, using expert clinical judgment as the gold standard. We compared the diagnostic accuracy of the Okazaki, Umehara, and ACR\/EULAR criteria in a cohort of 41 patients with suspected IgG4-RD. We assessed sensitivity, specificity, and positive and negative predictive values for each criterion, and conducted a separate analysis based on four IgG4-RD subtypes. Results: A total of 30 patients were confirmed to have IgG4-RD and 11 were identified as mimickers. The Umehara criteria demonstrated the highest sensitivity (83.33%), followed by the ACR\/EULAR 2019 (66.67%) and Okazaki (60.0%) criteria. All three criteria exhibited 100% specificity, with overall diagnostic accuracy ranging from 70% to 88%. The areas under the curve (AUC) were 0.917 (Umehara), 0.800 (Okazaki), and 0.833 (ACR\/EULAR 2019), indicating significant diagnostic effectiveness (p &lt; 0.000). Subtype analysis revealed that the Umehara and ACR\/EULAR 2019 criteria were more effective in diagnosing pancreato-hepato-biliary involvement (subtype 1), while the Okazaki and ACR\/EULAR 2019 criteria were more effective in diagnosing retroperitoneal fibrosis and\/or aortitis (subtype 2). Conclusions: Our study provides valuable insights into the diagnostic performance of the Okazaki, Umehara, and ACR\/EULAR criteria for a cohort of patients with suspected IgG4-RD. The Umehara criterion demonstrated the highest sensitivity, suggesting its potential utility for screening purposes, while all three criteria showed consistent specificity.<\/jats:p>","DOI":"10.3390\/diagnostics14222583","type":"journal-article","created":{"date-parts":[[2024,11,18]],"date-time":"2024-11-18T07:45:27Z","timestamp":1731915927000},"page":"2583","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Comparative Analysis of Classification Criteria in IgG4-Related Disease and Evaluating Diagnostic Accuracy from a Retrospective Cohort in Clinical Practice"],"prefix":"10.3390","volume":"14","author":[{"given":"Marta","family":"Lopez-Gomez","sequence":"first","affiliation":[{"name":"Rheumatology Department, Hospital Universitario Araba, 01009 Vitoria, Spain"},{"name":"Instituto de Investigaci\u00f3n Biom\u00e9dica BIORABA, Hospital Universitario Araba, 01009 Vitoria, Spain"},{"name":"Department of Medicine, Universitat Aut\u00f2noma de Barcelona (UAB), 08193 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8339-5420","authenticated-orcid":false,"given":"Patricia","family":"Moya-Alvarado","sequence":"additional","affiliation":[{"name":"Department of Medicine, Universitat Aut\u00f2noma de Barcelona (UAB), 08193 Barcelona, Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4972-9527","authenticated-orcid":false,"given":"Hye Sang","family":"Park","sequence":"additional","affiliation":[{"name":"Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar Concepci\u00f3n","family":"Mart\u00edn","sequence":"additional","affiliation":[{"name":"Gastroenterology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Calleja","sequence":"additional","affiliation":[{"name":"Immunology Department, Hospital de la Santa Creu i Sant Pau, IIB Sant Pau, UAB, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9883-9845","authenticated-orcid":false,"given":"Helena","family":"Codes-Mendez","sequence":"additional","affiliation":[{"name":"Department of Medicine, Universitat Aut\u00f2noma de Barcelona (UAB), 08193 Barcelona, Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berta","family":"Magallares","sequence":"additional","affiliation":[{"name":"Department of Medicine, Universitat Aut\u00f2noma de Barcelona (UAB), 08193 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5410-5807","authenticated-orcid":false,"given":"Iv\u00e1n","family":"Castellv\u00ed","sequence":"additional","affiliation":[{"name":"Department of Medicine, Universitat Aut\u00f2noma de Barcelona (UAB), 08193 Barcelona, Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4053-2821","authenticated-orcid":false,"given":"Antonio J.","family":"Barros-Membrilla","sequence":"additional","affiliation":[{"name":"Cardiology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2820-4801","authenticated-orcid":false,"given":"Ana","family":"Laiz","sequence":"additional","affiliation":[{"name":"Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6275-7699","authenticated-orcid":false,"given":"C\u00e9sar","family":"Diaz-Torn\u00e9","sequence":"additional","affiliation":[{"name":"Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9249-1983","authenticated-orcid":false,"given":"Luis","family":"Sainz","sequence":"additional","affiliation":[{"name":"Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain"},{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-0428-3399","authenticated-orcid":false,"given":"Julia","family":"Bern\u00e1rdez","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0126-158X","authenticated-orcid":false,"given":"Laura","family":"Mart\u00ednez-Martinez","sequence":"additional","affiliation":[{"name":"Immunology Department, Hospital de la Santa Creu i Sant Pau, IIB Sant 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