Sargin Gokhan, Senturk Taskin, Cildag Songul
Department of Rheumatology, Adnan Menderes University, Aydin, Turkey.
Int J Rheum Dis. 2018 May;21(5):1093-1097. doi: 10.1111/1756-185X.13311. Epub 2018 Apr 19.
Systemic sclerosis (SSc) has increased risk of morbidity and mortality due to pulmonary fibrosis, pulmonary arterial hypertension, renal crisis, infections and malignancies. Chemical exposure, smoking and cytotoxic drugs increase the malignancy risk in rheumatic diseases including SSc. We aim to evaluate characteristics, identify risk factors and mortality in SSc patients with malignancies.
One hundred and fifty-three patients with SSc (24 male, 129 female, with mean age of 56.4 ± 12.9 years) according to 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for SSc were enrolled in this study. Clinical, demographic, laboratory and radiological characteristics of the patients were evaluated by using SPSS 17.0. Chi-square test was used to compare qualitative data and descriptive statistics are shown as n (%).
Seven cases of cancer (three male, four female, with mean age of 51.2 ± 7.0 years) were identified. The tumor types included lung cancer, gastrointestinal tract cancer (gastric adenocarcinoma and gastric neuroendocrine tumor), myelodysplastic syndrome and malignant melanoma (eye and skin). Five (3.4%) died due to infection, cardiopulmonary involvement and renal crisis. Six of them had a diffuse cutaneous SSc clinical subtype, and the ratio for anti-Scl-70 was 71.4%. There was no significant relationship between age, sex, anti-Scl-70, pulmonary and cardiac involvement between cancer and non-cancer patients.
The malignancies may occur before, together or after the diagnosis of SSc. Both of these disorders have increased morbidity and mortality compared with the general population. Rational treatment, monitoring and reducing the risk factors may prevent the development of malignancy. Also, the clinical features, risk factors and prevalence ratios are similar to that reported by large cohorts.
系统性硬化症（SSc）因肺纤维化、肺动脉高压、肾危象、感染和恶性肿瘤而导致发病和死亡风险增加。化学物质暴露、吸烟和细胞毒性药物会增加包括SSc在内的风湿性疾病的恶性肿瘤风险。我们旨在评估SSc合并恶性肿瘤患者的特征、识别危险因素并分析死亡率。
根据2013年美国风湿病学会/欧洲抗风湿病联盟SSc分类标准，纳入153例SSc患者（男24例，女129例，平均年龄56.4±12.9岁）。使用SPSS 17.0评估患者的临床、人口统计学、实验室和放射学特征。采用卡方检验比较定性数据，描述性统计以n（%）表示。
共识别出7例癌症患者（男3例，女4例，平均年龄51.2±7.0岁）。肿瘤类型包括肺癌、胃肠道癌（胃腺癌和胃神经内分泌肿瘤）、骨髓增生异常综合征和恶性黑色素瘤（眼部和皮肤）。其中5例（3.4%）因感染、心肺受累和肾危象死亡。6例为弥漫性皮肤型SSc临床亚型，抗Scl-70阳性率为71.4%。癌症患者与非癌症患者在年龄、性别、抗Scl-70、肺部和心脏受累方面无显著相关性。
恶性肿瘤可能在SSc诊断之前、同时或之后发生。与普通人群相比，这两种疾病的发病率和死亡率均有所增加。合理的治疗、监测和降低危险因素可能预防恶性肿瘤的发生。此外，临床特征、危险因素和患病率与大型队列研究报告的相似。
