Purpose of review: As survival in systemic sclerosis (SSc) improves, research interest has shifted to the leading cause of non-SSc-related death, namely cancer, which accounts for over a third of non-SSc-related deaths. This review will provide an overview of the recent insights into the evolving relationship between SSc and cancer.
Recent findings: Recent studies confirm the increased risk of cancer in SSc compared with the general population (standardized incidence ratio 1.9-2.2) in particular the risk of breast, lung and skin cancer. This increased cancer risk, particularly occurring in close proximity to SSc onset, raises the novel concept of autoimmunity occurring as a direct immune response to the cancerous cells. We highlight the important role that SSc-specific autoantibodies may have in identifying these at-risk patients, prognostication and triaging those who may require tight surveillance and further cancer screening.
Summary: The knowledge will allow the development of future prospective studies evaluating clinically relevant and targeted cancer screening strategies for newly diagnosed SSc patients to optimize cancer detection while minimizing harms and costs from overscreening.