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Objectives The aims were to identify all incident adult cases of systemic autoimmune myopathies (SAMs) in the city of Leeds, UK, and to estimate the risk of cancer in SAMs as compared with the general population. Methods Cases of SAMs were ascertained by review of all muscle biopsy reports from the Neuropathology Laboratory. A review of medical records was undertaken for each case to review the clinical diagnosis and collect epidemiological data such as age, ethnicity, sex and comorbidities, including cancer. Leeds denominator population numbers were publicly obtainable. Results A total of 206 biopsy reports were identified and, after review, 50 incident cases were included in the study between June 2010 and January 2021. Of the 50 cases, 27 were male and 23 were female. The mean incidence rate of SAMs in Leeds throughout the study period was 7.42/1 000 000 person-years. The proportion of SAMs cases with a confirmed malignancy was 22%. Compared with the general population, the relative risk of cancer was significantly greater in the SAMs population (31.56; P &amp;lt; 0.01). Conclusions The incidence rate of SAMs in Leeds was consistent with data from previous literature; however, disagreement exists between different methods of SAMs case inclusion due to varying clinical criteria and definitions. SAMs are associated with an increased risk of cancer, but the pathogenesis of this relationship still requires investigating. This study supports the practice of malignancy screening and long-term surveillance in patients with SAMs.

Incidence of systemic autoimmune myopathies and their risk of cancer in Leeds, UK: an 11-year epidemiological study