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Epidemiology of Cancer in Systemic Sclerosis—Systematic Review and Meta-Analysis of Cancer Incidence, Predictors and Mortality*

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DOI: 10.4236/ojra.2013.34037    3,328 Downloads   5,206 Views  

ABSTRACT

Objectives: The study was conducted to improve our understanding of the epidemiology of cancer in systemic sclerosis (SSc) by evaluating the incidence, prevalence, relative risk of overall and site-specific malignancies, predictors and cancer-attributable mortality. Methods: MEDLINE, CINAHL, EMBASE and Cochrane Library (inception-May 2012) were searched. Estimates were combined using a random effects model. Consistency was evaluated using the I2 statistic. Results: 4876 citations were searched to identify 60 articles. The average incidence of malignancy in SSc was 14 cases/1000 person-years; the prevalence ranged between 4%-22%. Cancer was the leading cause of non-SSc related deaths with a mean of 38%. Overall SIR for all-site malignancy risk was 1.85 (95%CI 1.52, 2.25; I276%). There was a greater risk of lung (SIR 4.69, 95%CI 2.84, 7.75; I293%) and haematological (SIR 2.58, CI 95% 1.75, 3.81; I20%) malignancies, including non-Hodgkins lymphoma (SIR 2.55, 95%CI 1.40, 4.67; I20%). SSc patients were at a higher risk of leukemia (SIR 2.79, 95%CI 1.22, 6.37; I20%), malignant melanoma (SIR 2.92, 95%CI 1.76, 4.83; I235%), liver (SIR 4.75, 95%CI 3.09, 7.31; I20%), cervical (SIR 2.28, 95%CI 1.26, 4.09; I254%) and oropharyngeal (SIR 5.0, 95%CI 2.18, 11.47; I258%) cancers. Risk factors include a-RNAP I/III seropositivity, male sex, and late onset SSc. Smoking and longstanding interstitial lung disease increase the risk of lung cancer; Barretts esophagus and a positive family history of breast cancer, respectively, increase the risk of esophageal adenocarcinoma and breast cancer. Conclusions: SSc patients have a two-fold increase in all-site malignancy, and greater risk of lung and haematological malignancies that contribute significantly to mortality. Vigilance should be considered in SSc patients with risk factors for cancer.

Conflicts of Interest

The authors declare no conflicts of interest.

Cite this paper

T. Nevskaya, S. Chandran, A. Roos, C. Pasarikovski, A. Kron, C. Chau and S. Johnson, "Epidemiology of Cancer in Systemic Sclerosis—Systematic Review and Meta-Analysis of Cancer Incidence, Predictors and Mortality*," Open Journal of Rheumatology and Autoimmune Diseases, Vol. 3 No. 4, 2013, pp. 231-245. doi: 10.4236/ojra.2013.34037.

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