系统性硬化症（SSc）与癌症风险增加相关。我们的目的是评估队列中癌症的患病率，并探讨其与临床、免疫学和治疗特征之间可能的关联。我们对 SSc 患者的回顾性单中心队列研究记录了恶性肿瘤的流行和偶发病例，包括 SSc 3 年内诊断的病例发病（定义为癌症相关硬皮病），并在 SSc 诊断时、任何特定治疗之前，使用 RNA 和蛋白质免疫沉淀、蛋白质印迹、免疫印迹和 ELISA 寻找与临床特征和血清自身抗体分析的关联。在 290 名患者中SSc 患者的癌症总体患病率为 20%，其中 8% 的病例是与癌症相关的硬皮病。这两种情况在老年患者和抗 Ro52 或抗 U3-RNP 阳性的患者中更为常见。癌症相关硬皮病在抗着丝粒 (ACA) 和抗拓扑异构酶 1 (TOPO1) 抗体阴性的患者中明显更为常见，特别是在弥漫性 SSc 的情况下。免疫抑制剂与癌症没有显着相关性。 ACA、TOPO1 和抗 RNA 聚合酶 III 抗体三阴性的患者患乳腺癌的风险显着较高。对于弥漫性 SSc、发病年龄增加且没有经典 SSc 相关自身抗体的患者，癌症监测应特别小心。© 作者（或其雇主）2024。根据 CC BY-NC 允许重复使用。禁止商业再利用。请参阅权利和权限。英国医学杂志出版。

Systemic sclerosis (SSc) is associated with an increased risk of cancer. We aimed to assess the prevalence of cancer in our cohort and to explore possible associations with clinical, immunological and treatment characteristics.Our retrospective monocentric cohort study of patients with SSc recorded prevalent and incident cases of malignancy, including those diagnosed within 3 years of the SSc onset (defined as cancer-associated scleroderma) and sought associations with the clinical characteristics and the serum autoantibody profiling performed using RNA and protein immunoprecipitation, Western-blot, immunoblot and ELISA at the time of SSc diagnosis, prior to any specific treatment.Among 290 patients with SSc, the overall prevalence of cancer was 20%, with 8% of cases being cancer-associated scleroderma. Both conditions were more frequent in elderly patients and in patients with positive anti-Ro52 or anti-U3-RNP. Cancer-associated scleroderma was significantly more prevalent among patients negative for both anti-centromere (ACA) and anti-topoisomerase-1 (TOPO1) antibodies, especially in the case of diffuse SSc. Immunosuppressants were not significantly associated with cancer. Patients triple negative for ACA, TOPO1 and anti-RNA polymerase III antibodies had a significantly higher risk of breast cancer.Cancer surveillance should be particularly careful in patients with diffuse SSc, increased age at disease onset and without classical SSc-related autoantibodies.© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.