Incidence and the Risk Factor of Malignancy in Japanese Patients with Systemic Sclerosis

  • Hashimoto Atsushi
    Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Japan Department of Rheumatology, Sagamihara National Hospital, National Hospital Organization, Japan
  • Arinuma Yoshiyuki
    Department of Rheumatology, Sagamihara National Hospital, National Hospital Organization, Japan
  • Nagai Tatsuo
    Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Japan
  • Tanaka Sumiaki
    Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Japan
  • Matsui Toshihiro
    Department of Rheumatology, Sagamihara National Hospital, National Hospital Organization, Japan
  • Tohma Shigeto
    Department of Rheumatology, Clinical Research Center for Allergy and Rheumatology, Sagamihara National Hospital, National Hospital Organization, Japan
  • Endo Hirahito
    Division of Rheumatology, Department of Internal Medicine, Toho University School of Medicine, Japan
  • Hirohata Shunsei
    Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Japan

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Abstract

Objective To evaluate the incidence and risk factors for malignancy in Japanese patients with systemic sclerosis (SSc).<br> Methods 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).<br> Results 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).<br> Conclusion Lung cancer was significantly frequent in SSc patients, which could develop on the basis of complicated ILD.<br>

Journal

  • Internal Medicine

    Internal Medicine 51 (13), 1683-1688, 2012

    The Japanese Society of Internal Medicine

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