Qian Junyan, He Chengmei, Li Ya, Peng Linyi, Yang Yunjiao, Xu Dong, Fei Yunyun, Zhang Wen, Zhao Yan, Dong Yi, Li Yongzhe, Zhang Xuan, Chen Hua, Yang Yanlei, Chen Zhilei, Liu Suying, Wang Li, Zhang Fengchun
Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology; State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH); Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China.
Department of Clinical Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China.
Ther Adv Musculoskelet Dis. 2021 Jun 23;13:1759720X211020179. doi: 10.1177/1759720X211020179. eCollection 2021.
To investigate the long-term survival of patients with primary Sjögren's syndrome (pSS) in China.
Patients with pSS who fulfilled the 2002 American-European Consensus Group classification criteria were prospectively enrolled from 2004 to 2011. Their baseline clinical, laboratory, and therapeutic data were collected. The primary endpoint was all-cause death by January 2018. The standard mortality ratio (SMR) was calculated by comparing with age-matched and sex-matched mortality data of the general population. Kaplan-Meier curves were obtained by time-to-event analysis. Univariate and multivariate Cox hazards regression analyses were performed to identify risk factors for mortality.
A total of 1054 patients were enrolled and 834 patients were followed up for a median of 94.8 months, with 48 confirmed deaths. The total SMR was 3.63 [95% confidence interval (CI) 2.60-4.66]. The 3-, 5-, and 10-year survival rates were 98.4%, 97.5%, and 92.9%, respectively. Infection, malignancy, and respiratory failure were the top three causes of mortality. We identified male sex [hazard ratio (HR) = 3.00, 95% CI 1.23-7.31], age at diagnosis ⩾50 years of age (HR = 7.69, 95% CI 3.01-19.62), thrombocytopenia (HR = 1.93, 95% CI 1.01-3.72), and interstitial lung disease (HR = 5.96, 95% CI 2.24-15.82) as the independent prognostic factors of death.
The mortality rates of Chinese patients with pSS are higher than those of the general population. Male patients of elder age at diagnosis complicated with thrombocytopenia and interstitial lung disease might be suggestive for poorer survival and require closer follow up.
调查中国原发性干燥综合征（pSS）患者的长期生存率。
前瞻性纳入2004年至2011年期间符合2002年欧美共识小组分类标准的pSS患者。收集其基线临床、实验室和治疗数据。主要终点是截至2018年1月的全因死亡。通过与年龄和性别匹配的普通人群死亡率数据进行比较来计算标准化死亡率（SMR）。通过事件发生时间分析获得Kaplan-Meier曲线。进行单因素和多因素Cox风险回归分析以确定死亡的危险因素。
共纳入1054例患者，834例患者接受了中位时间为94.8个月的随访，其中48例确诊死亡。总SMR为3.63[95%置信区间（CI）2.60 - 4.66]。3年、5年和10年生存率分别为98.4%、97.5%和92.9%。感染、恶性肿瘤和呼吸衰竭是死亡的三大主要原因。我们确定男性[风险比（HR）= 3.00，95% CI 1.23 - 7.31]、诊断时年龄≥50岁（HR = 7.69，95% CI 3.01 - 19.62）、血小板减少（HR = 1.93，95% CI 1.01 - 3.72）和间质性肺病（HR = 5.96，95% CI 2.24 - 15.82）为死亡的独立预后因素。
中国pSS患者的死亡率高于普通人群。诊断时年龄较大的男性患者合并血小板减少和间质性肺病可能提示生存率较差，需要更密切的随访。
