Background Systemic sclerosis (Scleroderma) is a rare, immune-mediated, complex disorder characterized by extensive fibrosis and vascular alterations. A higher incidence of cancer - especially lung, breast and hematological cancer- in scleroderma patients compared to the general population has been suggested by several observational studies, reporting, however, different estimates.

Objectives The present study performed a systematic review and meta-analysis to definitely assess this relevant issue.

Methods We searched Medline for all original articles of observational studies on cancer incidence in scleroderma patients without language restriction published up to December 2010. Two independent authors reviewed all titles/abstracts and retrieved detailed full-text of potentially relevant articles to identify studies according to predefined selection criteria. Summary estimates were derived using random effects model and reported as relative risk (RR). Publication bias was evaluated by trim- and fill- analysis.

Results From 1102 articles initially identified, 15 original studies, involving more than 5000 patients, were included in the present review. Compared to the general population, the summary RR to develop all invasive cancers in scleroderma patients was 1.81 (95% CI, 1.40-2.34). The results for selected cancer sites indicated a strong association with lung cancer (RR 4.4; 95% CI, 1.86-10.31), and a significant increased risk also for haematological neoplasms (RRs 2.1; 95%, CI 1.35-3.14). The relation with breast cancer, suggested in some previous epidemiological studies, was not confirmed (RRs 1.01; 95% CI, 0.81-1.26).

Conclusions The present meta-analysis, the first on scleroderma and cancer risk, provides definite evidence of a significant association between scleroderma and cancer.

Disclosure of Interest None Declared