Abstract
Objective To investigate the risk of mortality due to malignancy in patients with primary Sjögren's Syndrome(pSS).Methods From May 2016 to January 2022,patients registered in the Chinese Rheumatism Data Center(CRDC)who fulfilled the 2002 American-European Consensus Group or the 2016 A-merican College of Rheumatology/European League Against Rheumatism classification criteria for pSS were en-rolled.Clinical data,including basic demographic features,disease duration,clinical manifestations,au-toantibodies,serum complement levels,and EULAR Sjögren's Syndrome Disease Activity Index(ESSDAI),were analyzed retrospectively to assess the risk and influencing factors of death due to solid tumors or hematolog-ic malignancies.Results The median follow-up duration for the 8,932 pSS patients was 48.0(32.1,72.0)months.During this period,56 patients died due to malignant tumors,with a crude tumor mortality rate of 0.6％.The standardized mortality ratio(SMR)was 0.841,95％CI:0.636-1.093,with a standardized tumor mortality rate of 0.5％.Among these,46 patients(82.1％)died due to solid tumors,and 10(17.9％)due to hematologic malignancies.Factors associated with an increased risk of death due to malignant tumors included aging(SHR=1.056,95％CI:1.028-1.085,P＜0.001),thrombocytopenia(SHR=3.982,95％CI:2.123-7.471,P＜0.001),and high pulmonary activity based on ESSDAI scores(SHR=5.291,95％CI:1.827-15.328,P=0.002).Immunosuppressive therapy was identified as a protective factor(SHR=0.348,95％CI:0.164-0.741,P=0.006).Lung cancer(16,28.6％)was the leading cause of death in solid tumors.The crude mortality rate of lung cancer was 0.2％,with an SMR of 1.284,95％CI:0.734-2.085,and a standardized tumor mortality rate of 0.2％.Risk factors for death due to lung cancer included male gender(HR=8.356,95％CI:2.676-26.100,P＜0.001),aging(HR=1.102,95％CI:1.053-1.153,P＜0.001),and high pulmonary activity lesions in the ESSDAI score(2 vs.0 points:HR=7.041,95％CI:2.233-22.208,P=0.001;3 vs.0 points:HR=17.349,95％CI:3.593-83.764,P＜0.001).Hydroxychloroquine was a protective factor(HR=0.213,95％CI:0.069-0.663,P=0.008).Conclusion The risk of death due to malignant tumors in pSS patients does not significantly dif-fer from the general population.Aging,thrombocytopenia,and high pulmonary activity based on ESSDAI scores are risk factors for malignant tumor-related death events.Immunosuppressive therapy can decrease the overall risk of tumor-related mortality.Risk factors for death due to lung cancer include male,aging,and high pulmonary ac-tivity based on the ESSDAI score,while hydroxychloroquine reduces the risk of death due to lung cancer.关键词
原发性干燥综合征/恶性肿瘤/死亡风险Key words
primary Sjögren's syndrome/malignancy/mortality risk
