背景:系统性硬化症(SSc)患者患恶性肿瘤的风险增加。在这项研究中,我们的目的是分析癌症的患病率,风险因素和对总生存率的影响。
方法:我们分析了临床(癌症史,毒性暴露,器官受累),在2004年1月至2017年12月期间,对SSc患者单中心队列的免疫学和治疗数据进行了随访.
结果:纳入200名SSc患者。在后续行动中,21例患者(10%)被诊断为恶性肿瘤.潜在的恶性肿瘤是乳腺癌(n=6,28%),肺癌(n=6,28%),结直肠(结肠腺癌,阑尾类癌),卵巢和子宫颈,黑色素瘤,肾和甲状腺乳头状癌(各1例)。首次就诊与癌症诊断之间的中位时间为4[2-10]年。SSc癌症患者的总生存期与无癌症患者没有显着差异。在第一个四分位数的中位生存期(75%)中,癌症患者为12年,非癌症患者为11.6年(P=0.9)。肾硬皮病危象史(HR10.99,IC95%[1.95-62.07];P=0.006)和抗拓扑异构酶I抗体的存在(HR5.5,IC95%[1.40-21.67];P=0.01)与癌症风险增加相关,而胃食管反流的存在与癌症的发生呈负相关(HR0.22,IC95%[0.056-0.867];P=0.03).
结论:在这项单中心研究中,肾硬皮病危象病史和抗拓扑异构酶I抗体阳性与SSc患者癌症风险增加相关。
BACKGROUND: Patients with systemic sclerosis (SSc) have an increased risk of malignancy. In this study, we aimed to analyze the prevalence of cancer, the risk factors and the impact on overall survival.
METHODS: We analyzed clinical (history of cancer, toxic exposition, organ involvement), immunological and treatment data in a monocentric cohort of SSc patients followed between January 2004 and December 2017.
RESULTS: Two hundred and ten patients with SSc were included. During the follow-up, twenty-one patients (10 %) were diagnosed with malignancies. The underlying malignancies were breast adenocarcinoma (n=6, 28%), lung cancer (n=6, 28%), colorectal (colic adenocarcinoma, carcinoid tumor of the appendix), ovarian and cervix uteri, melanoma, kidney and papillary thyroid carcinoma (one of each). The median time between the first visit and the diagnosis of cancer was 4 [2-10] years. The overall survival in SSc patients with cancer was not significantly different from patients without cancer, with median survival during the first quartile (75%) at 12 years for patients with cancer and 11.6 years for those without cancer (P=0.9). The history of renal scleroderma crisis (HR 10.99, IC95% [1.95-62.07]; P=0.006) and the presence of anti-topoisomerase I antibodies (HR 5.5, IC95% [1.40-21.67]; P=0.01) were associated with an increased risk of cancer, whereas the presence of gastroesophageal reflux was inversely associated with the cancer occurrence (HR 0.22, IC95% [0.056-0.867]; P=0.03).
CONCLUSIONS: The history of renal scleroderma crisis and the positivity of anti-topoisomerase I antibodies were associated with an increased risk of cancer in SSc patients in this monocentric study.