Department of Preventive Medicine, College of Medicine, Korea University, Seoul, Republic of Korea.
Department of Public Health, Graduate School, Korea University, Seoul, Republic of Korea; Health Insurance Review and Assessment Service, Republic of Korea.
Semin Arthritis Rheum. 2017 Oct;47(2):222-227. doi: 10.1016/j.semarthrit.2017.03.004. Epub 2017 Mar 8.
The objective of this study was to investigate the epidemiological features of primary Sjögren's syndrome (pSS) in Korea at a national level, including the incidence, mortality, and causes of death.
We used a national, population-based registry database called the Rare Intractable Disease Registration Program from the Health Insurance Review and Assessment Service to obtain pSS patient data for the period between 2010 and 2014. pSS was diagnosed by a physician based on uniform criteria. We also used data from Statistics Korea to confirm the mortality and causes of death.
Between 2010 and 2014, the total number of patients newly diagnosed with pSS was 5891, resulting in an annual incidence of 2.34 per 100,000 individuals. The female-to-male ratio was 14.5:1. A total of 114 pSS patients died during the study period. The overall survival rate of pSS patients was 99.0%, and the 1-year, 2-year, and 5-year survival rates were 98.7%, 98.1%, and 97.1%, respectively, and the standardized mortality ratio (SMR) was 1.47 (2.14 for males and 1.35 for females). The most common causes of death were respiratory disease (n = 25; 21.9%) followed by circulatory diseases (n = 21; 18.4%), musculoskeletal connective tissue diseases (n = 21; 18.4%), and cancer (n=19; 16.7%).
The national incidence of pSS in Korea presented in this study was lower in comparison with reports from other countries. However, the mortality rate was significantly higher than the corresponding values in the age- and gender-matched general population. The higher mortality in pSS patients is attributable to respiratory diseases and lung cancer.
本研究旨在调查韩国全国范围内原发性干燥综合征（pSS）的流行病学特征，包括发病率、死亡率和死因。
我们使用了一个名为健康保险审查与评估服务的罕见疑难病登记项目的全国性人群登记数据库，获取了 2010 年至 2014 年期间 pSS 患者的数据。pSS 的诊断由医生根据统一标准进行。我们还使用了韩国统计局的数据来确认死亡率和死因。
2010 年至 2014 年期间，新诊断为 pSS 的患者总数为 5891 例，发病率为每年每 10 万人 2.34 例。男女比例为 14.5:1。在研究期间，共有 114 例 pSS 患者死亡。pSS 患者的总生存率为 99.0%，1 年、2 年和 5 年生存率分别为 98.7%、98.1%和 97.1%，标准化死亡率比（SMR）为 1.47（男性为 2.14，女性为 1.35）。最常见的死因是呼吸系统疾病（n=25；21.9%），其次是循环系统疾病（n=21；18.4%）、肌肉骨骼结缔组织疾病（n=21；18.4%）和癌症（n=19；16.7%）。
与其他国家的报告相比，本研究中韩国的全国性 pSS 发病率较低。然而，死亡率明显高于相应年龄和性别匹配的一般人群。pSS 患者的高死亡率归因于呼吸系统疾病和肺癌。
