Servicio de Reumatología y Metabolismo Óseo, Consorcio Hospital General Universitario, Valencia, Spain; Escuela de Doctorado, Universidad Católica de Valencia San Vicente Mártir (UCV), Valencia, Spain.
Servicio de Reumatología y Metabolismo Óseo, Consorcio Hospital General Universitario, Valencia, Spain; Escuela de Doctorado, Universidad Católica de Valencia San Vicente Mártir (UCV), Valencia, Spain.
Med Clin (Barc). 2024 Sep 13;163(5):217-223. doi: 10.1016/j.medcli.2024.03.026. Epub 2024 Jun 6.
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.
IgG4 相关疾病（IgG4-RD）是一种罕见的、系统性免疫介导的纤维炎症性疾病，病因和发病机制尚不清楚，可能影响多个器官。它具有常见的临床、放射学和血清学特征。本研究旨在比较最新的两种 IgG4-RD 分类和诊断标准：Umehara-Okazaki 2011 标准和 ACR/EULAR 2019 标准。
在 2010 年 1 月至 2023 年 7 月期间，我们在两个中心进行了一项回顾性横断面研究，纳入了来自不同医院科室的疑似 IgG4-RD 患者。排除最终诊断为其他病理学的患者。使用 Umehara-Okazaki 2011 标准和 ACR/EULAR 2019 标准评估其余疑似 IgG4-RD 病例。
在 34 例临床诊断为 IgG4-RD 的患者中，Umehara-Okazaki 2011 标准分类为 20 例：5 例为明确，7 例为可能，8 例为可能病例。应用 ACR/EULAR 2019 标准对同一队列进行诊断，结果为 9 例。值得注意的是，腹膜后纤维化和主动脉炎是最常见的表现形式，在 2011 年和 2019 年标准下分别占病例的 25%和 22.2%。
更新更严格的 ACR/EULAR 2019 标准侧重于组织病理学、各种表现形式和分析数据，能够更准确地对患者进行分类。
