IgG4-related disease: Effectiveness evaluation through Umehara–Okazaki 2011 and ACR/EULAR 2019 diagnostic criteria

• DOI: 10.1016/j.medcli.2024.03.026
• Publication Date: 2024-06-06T11:29:56Z
• Authors: Pablo Martínez Ca...; Jorge Juan Fragío...; Roxana González M...; Fernando López Gu...; Javier Loricera G...; Ricardo Blanco Al...; Cristina Campos F...
• Keywords: Okazaki fragments; Interquartile range; IgG4-Related Disease; Presentation (obstetrics); Etiology

IgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting multiple organs. It presents with common clinical, radiological, and serological characteristics. This study aims to compare the latest two IgG4-RD classification and diagnostic criteria: Umehara-Okazaki 2011 and ACR/EULAR 2019.In a retrospective cross-sectional study conducted across two centers from January 2010 to July 2023, we included patients suspected of having IgG4-RD from various hospital departments. Patients finally diagnosed with other pathologies were excluded. The remaining suspected IgG4-RD cases were evaluated using both Umehara-Okazaki 2011 and ACR/EULAR 2019 criteria.Out of 34 patients with a clinical diagnosis of IgG4-RD, the Umehara-Okazaki 2011 classified 20 patients: 5 as definitive, 7 as probable, and 8 as possible cases. Applying the ACR/EULAR 2019 criteria to the same cohort resulted in the diagnosis of 9 patients. Notably, retroperitoneal fibrosis and aortitis were the most prevalent form of presentation, accounting for 25% and 22.2% of cases classified under the 2011 and 2019 criteria, respectively.The more recent and stringent ACR/EULAR 2019 criteria focus on histopathology, various forms of presentation, and analytical data, allow for a more accurate classification of patients.