Hashimoto Atsushi, Arinuma Yoshiyuki, Nagai Tatsuo, Tanaka Sumiaki, Matsui Toshihiro, Tohma Shigeto, Endo Hirahito, Hirohata Shunsei
Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Japan.
Intern Med. 2012;51(13):1683-8. doi: 10.2169/internalmedicine.51.7686. Epub 2012 Jul 1.
To evaluate the incidence and risk factors for malignancy in Japanese patients with systemic sclerosis (SSc).
A cohort of 405 Japanese patients with SSc who visited Kitasato University hospital between 1973 and 2008 was analyzed retrospectively until the end of 2009. The incidence of malignancy was compared with that of the general population, with calculation of the standardized incidence ratio (SIR) and 95% confidence interval (CI).
The cohort represented 4,787 person-years of total disease duration after the diagnosis of SSc. Of 27 malignancies clinically found, lung cancer (n=10, 37%), especially adenocarcinoma, was the most frequent, followed by breast (n=4, 15%) or gastric (n=3, 11%) cancer. SSc patients with overlapping CTD tended to have less malignancy. Multivariable analysis revealed heart involvement of SSc as a significant risk factor for breast cancer (RR 8.2, 95% CI 1.2-72.8). Other than gastric cancer, the calculated SIRs of malignancies in SSc patients were above 1 (SIR of overall malignancy 1.24, 95% CI 0.77-1.71), even though only lung cancer had a significantly elevated incidence (SIR 5.73, 95% CI 2.18-9.29). Every patient with lung cancer had interstitial lung disease (ILD) and every autopsy performed on patients with lung cancer found a primary lesion of lung cancer in their ILD lesion (n=4).
Lung cancer was significantly frequent in SSc patients, which could develop on the basis of complicated ILD.
评估日本系统性硬化症（SSc）患者发生恶性肿瘤的发生率及危险因素。
回顾性分析1973年至2008年间就诊于北里大学医院的405例日本SSc患者，直至2009年底。将恶性肿瘤的发生率与普通人群进行比较，计算标准化发病比（SIR）及95%置信区间（CI）。
该队列在SSc诊断后共代表4787人年的疾病总病程。临床发现的27例恶性肿瘤中，肺癌（n = 10，37%）最为常见，尤其是腺癌，其次是乳腺癌（n = 4，15%）或胃癌（n = 3，11%）。合并重叠性结缔组织病（CTD）的SSc患者发生恶性肿瘤的倾向较低。多变量分析显示，SSc的心脏受累是乳腺癌的一个显著危险因素（相对危险度8.2，95% CI 1.2 - 72.8）。除胃癌外，SSc患者恶性肿瘤的计算SIR均高于1（总体恶性肿瘤的SIR为1.24，95% CI 0.77 - 1.71），尽管只有肺癌的发病率显著升高（SIR 5.73，95% CI 2.18 - 9.29）。每例肺癌患者均患有间质性肺疾病（ILD），对肺癌患者进行的每例尸检均在其ILD病变中发现肺癌原发灶（n = 4）。
SSc患者中肺癌明显常见，可能在合并ILD的基础上发生。
