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The prevalence was 32 per million inhabitants, 19 per million women and 45 per million men, by December 31, 2017. Mean incidence was 2.5 per million inhabitants and year. Mean age at symptom onset was 64.4\u2009years with quadriceps weakness being the most common presenting symptom followed by finger flexor weakness. Dysphagia was a common presenting symptom being more frequent in women (23%) than men (10%) and was during the disease course reported in 74% of men and 84% of women. Seventy\u2010three patients were deceased, with a mean survival of 14\u2009years from symptom onset. Survival rates from both diagnosis date and symptom onset were decreased compared to the matched population.<\/jats:p><jats:p>Twenty\u2010one percent of the patients had an additional autoimmune disease. A cross\u2010sectional analysis of autoantibodies in 50 patients and 28 matched controls showed autoantibodies to cytosolic 5\u2032\u2010nucleotidase 1A in 40% of the patients and 3.6% of controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Inclusion body myositis is an autoimmune disease with decreased survival rate and with marked sex differences in both prevalence and clinical manifestations. 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