沙特原发性干燥综合征患者的疾病表型和诊断延迟：一项探索性横断面研究。
Disease phenotype and diagnostic delay in Saudi patients with primary Sjögren's syndrome: An exploratory cross-sectional study.
机构信息
From the Rheumatology Division (Omair), Pulmonary Division (AlHamad, Paramasivam) Department of Medicine, from the Department of Zoology (AlQahtani, Daghestani), College of Medicine, from the Department of Statistics and Operations Research, College of Sciences (Tashkandy), Central Laboratory (Othman), King Saud University; from the Rheumatology Division, Department of Medicine (AlShahrani), Diriyah General Hospital, Ministry of Health; from the Department of Clinical Science, College of Medicine (AlEnzi), Princess Nourah bint Abdulrahman University, Riyadh, Kingdom of Saudi Arabia; and from the Department of Clinical Sciences, College of Medicine (Halwani), Sharjah Institute for Medical Research, University of Sharjah, Sharjah, United Arab Emirates.
出版信息
Saudi Med J. 2021 Apr;42(4):405-410. doi: 10.15537/smj.2021.42.4.20200767.
OBJECTIVES
To describe primary Sjögren's syndrome (pSS) cohort in Saudi Arabiain view in of clinical/serological/histopathological phentotype, and, diagnostic delay.
METHODS
A cross-sectional study conducted between October 2018 and May 2019. Diagnostic delay was calculated from symptoms onset to clinical diagnosis. The European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) and EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI) were calculated.
RESULTS
Forty-one patients were included in the study. There were predominantly females (78%) with a mean (±SD) age of 58.76±12.7 and disease duration of 4.6±2.28 years. The mean diagnostic delay was 2.2±2.4 (range 1-11) years. Minor salivary gland biopsy was performed on 38 (92.7%) patients with a mean focus score of 2.3± 1.2 points. Interstitial lung disease and arthritis were the most common extra-glandular manifestations (EGM) affecting 27 (65.9%) patients for both. The mean ESSDAI was 9.95±7.73 and ESSPRI was 5.17±2.4.
CONCLUSION
Saudi primary Sjogren's syndrome patients have a high prevalence of EGM predominantly arthritis and ILD. The diagnostic delay is variable in our cohort.
目的
描述沙特阿拉伯原发性干燥综合征 (pSS) 患者的临床/血清学/组织病理学表型以及诊断延迟情况。
方法
本研究为 2018 年 10 月至 2019 年 5 月进行的一项横断面研究。从症状出现到临床诊断的时间被计算为诊断延迟。计算了欧洲抗风湿病联盟 (EULAR) 干燥综合征疾病活动指数 (ESSDAI) 和 EULAR 干燥综合征患者报告指数 (ESSPRI)。
结果
本研究共纳入 41 例患者，其中女性居多 (78%)，平均年龄 (±标准差) 为 58.76±12.7 岁，疾病病程为 4.6±2.28 年。平均诊断延迟为 2.2±2.4 (范围 1-11) 年。38 例 (92.7%) 患者进行了唾液腺活检，平均活检焦点评分 2.3±1.2 分。间质性肺疾病和关节炎是最常见的腺外表现 (EGM)，均影响 27 例 (65.9%) 患者。平均 ESSDAI 为 9.95±7.73，ESSPRI 为 5.17±2.4。
结论
沙特原发性干燥综合征患者 EGM 的发生率较高，主要为关节炎和间质性肺病。本研究队列中的诊断延迟情况各不相同。
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