Epidemiology Of Cancer In Systemic Sclerosis. Systematic Review and Meta-Analysis Of Cancer Incidence, Predictors and Mortality

Tatiana Nevskaya¹, Shelly Chandran², Adrienne M. Roos¹, Christopher Pasarikovski¹, Amie T. Kron³, Cathy Chau⁴ and Sindhu R. Johnson⁵, ¹Medicine, Toronto Scleroderma Research Program, Toronto Western Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, ON, Canada, ²Medicine, Toronto Scleroderma Research Program, Toronto Western Hospital, University of Toronto, Toronto, ON, Canada, ³Medicine, Toronto Scleroderma Program, Toronto Western Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, ON, Canada, ⁴Medicine, Toronto Scleroderma Program, Toronto Western Hospital, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ⁵Medicine, Toronto Western Hospital, Toronto General Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, ON, Canada

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Malignancy and risk

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II

Session Type: Abstract Submissions (ACR)

Background/Purpose:

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 in comparison with the general population, 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 I²statistic.

Results:

4,876 citations were searched to identify 59 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-Hodgkin’s 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%), 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 positivity, male sex, and late onset SSc. Smoking and longstanding interstitial lung disease (ILD) increase the risk of lung cancer; longstanding gastroesophageal reflux disease with Barrett’s esophagus and a positive family history of breast cancer, respectively, increase the risk of esophageal adenocarcinoma and breast cancer.

Conclusion:

SSc patients have a two-fold increase in malignancy, and greater risk of lung and haematological malignancies that contribute significantly to mortality. Vigilance should be considered in SSc patients with a-RNAP I/III antibodies, male sex, smokers, late disease onset, a positive family history of breast cancer, long duration of ILD, Barrett’s esophagus.

Disclosure:

T. Nevskaya,
None;

S. Chandran,
None;

A. M. Roos,
None;

C. Pasarikovski,
None;

A. T. Kron,
None;

C. Chau,
None;

S. R. Johnson,
None.

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