Aim: This study aimed to evaluate the long-term survival, causes of death, and prognostic factors in Chinese patients with primary Sjogren syndrome (pSS). Methods: We included patients with pSS registered in the Chinese Rheumatism Data Centre between May 2016 and December 2021, and collected baseline clinical, laboratory, and treatment data. Survival and standard mortality rates were calculated using general population mortality data. Factors related to mortality were identified using Cox proportional hazards regression. Results: Among the 8588 patients included, 274 died during a median follow-up of 4.0 years. The overall standardized mortality ratio was 1.61 (95% CI: 1.43-1.81). Overall survival rates were 98.2% at 5 years and 93.8% at 10 years. The predominant causes of death were comorbidities, including cardiovascular diseases, tumors, and infections, while the most frequent pSS-related causes of death were interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Male sex, older age, ILD, PAH, and high EULAR Sjogren's syndrome disease activity index (ESSDAI), thrombocytopenia, anemia, high immunoglobulin A (IgA) level, and glucocorticoid treatment independently increased the mortality risk, while using hydroxychloroquine was a protective factor. Conclusion: Mortality rates have significantly increased in Chinese patients with pSS. Comorbidities, rather than pSS-related organ damage, were the main causes of death. All-cause mortality was associated with male sex, older age, ILD, PAH, high ESSDAI, thrombocytopenia, anemia, high IgA level, and glucocorticoid treatment, whereas hydroxychloroquine use might improve the long-term survival.
基金:
Chinese National Key Technology RD Program [2017YFC0907601, 2017YFC0907605]; Chinese National Key Technology R&D Program, Ministry of Science and Technology [2021-I2M-1-005]; CAMS Innovation Fund for Medical Sciences (CIFMS) [2022-PUMCH-B-013]; National High-Level Hospital Clinical Research Funding
第一作者机构:[1]Peking Union Med Coll Hosp, Dept Rheumatol & Clin Immunol, Shuaifuyuan 1, Beijing 100730, Peoples R China[2]Chinese Acad Med Sci, Peking Union Med Coll, Beijing, Peoples R China[3]Peking Union Med Coll Hosp, Natl Clin Res Ctr Dermatol & Immunol Dis NCRC DID, State Key Lab Complex Severe & Rare Dis, Minist Sci & Technol, Beijing, Peoples R China[4]Minist Educ, Key Lab Rheumatol & Clin Immunol, Beijing, Peoples R China
共同第一作者:
通讯作者:
通讯机构:[1]Peking Union Med Coll Hosp, Dept Rheumatol & Clin Immunol, Shuaifuyuan 1, Beijing 100730, Peoples R China[2]Chinese Acad Med Sci, Peking Union Med Coll, Beijing, Peoples R China[3]Peking Union Med Coll Hosp, Natl Clin Res Ctr Dermatol & Immunol Dis NCRC DID, State Key Lab Complex Severe & Rare Dis, Minist Sci & Technol, Beijing, Peoples R China[4]Minist Educ, Key Lab Rheumatol & Clin Immunol, Beijing, Peoples R China
推荐引用方式(GB/T 7714):
Li Yueting,Qiao Lin,Xu Jian,et al.Long-term survival analysis of patients with primary Sjogren's syndrome in China: A multicenter retrospective cohort study[J].INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES.2024,27(9):doi:10.1111/1756-185X.15284.
APA:
Li, Yueting,Qiao, Lin,Xu, Jian,Duan, Xinwang,Wang, Yongfu...&Zhao, Yan.(2024).Long-term survival analysis of patients with primary Sjogren's syndrome in China: A multicenter retrospective cohort study.INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES,27,(9)
MLA:
Li, Yueting,et al."Long-term survival analysis of patients with primary Sjogren's syndrome in China: A multicenter retrospective cohort study".INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES 27..9(2024)