Inst. De Investigaciones En Ciencias De La Salud (INICSA), Universidad Nacional de Córdoba (UNC), Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Córdoba, and Inst. Universitario de Ciencias Biomédicas de Córdoba (IUCBC), Argentina.
Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey.
Clin Exp Rheumatol. 2019 May-Jun;37 Suppl 118(3):97-106. Epub 2019 Aug 28.
To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients.
The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded.
Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons).
More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.
在一个大型国际多民族原发性干燥综合征（SS）患者队列中，根据 ESSDAI 分类以外的标准分析原发性 SS 的频率和系统性表现特征。
大数据干燥综合征项目联盟是一个国际多中心注册机构，基于全球范围内的数据共享和对来自五大洲临床研究中领先中心的现有临床 SS 数据库的合作合并。根据先前的研究，定义了 26 个不包括在 ESSDAI 分类中的器官特异性系统性特征列表；这些特征被回顾性记录。
6331 例患者[5917 例女性，诊断时平均年龄 52 岁，主要为白人（86.3%）]中提供了有关非 ESSDAI 特征的信息。共有 1641 例（26%）患者存在至少一种 ESSDAI 系统性特征。心血管表现是本患者群体中报告的最常见的非 ESSDAI 特征器官特异性组（占总队列的 17%），其中报告有雷诺现象（15%）。存在非 ESSDAI 特征所致系统性疾病的患者口干（90.7%与 94.1%，p<0.001）和阳性唾液腺活检（86.7%与 89%，p=0.033）的频率较低，抗 Ro/SSA 抗体（74.7%与 68.7%，p<0.001）、抗 La/SSB 抗体（44.5%与 40.4%，p=0.004）、抗核抗体（ANA）（82.7%与 79.5%，p=0.006）、C3 水平低（17.4%与 9.7%，p<0.001）、C4 水平低（14.4%与 9.6%，p<0.001）和阳性血清冷球蛋白（8.6%与 5.5%，p=0.001）的频率较高。与无这些特征的患者相比，存在非 ESSDAI 系统性疾病的患者的系统性疾病活动度通过 ESSDAI、clinESSDAI 和 DAS 测量均较高（所有比较的 p<0.001）。
超过四分之一的原发性 SS 患者可能存在目前未包含在 ESSDAI 分类中的系统性表现，存在广泛的心血管、消化、呼吸、神经、眼部、耳鼻喉（耳、鼻、喉）、皮肤和泌尿系统特征，这些特征增加了疾病系统性表型的范围。然而，除了雷诺现象之外，这些非 ESSDAI 特征中的每一个的单独频率都非常低。
