Epidemiologic study of clinically amyopathic dermatomyositis and anti-melanoma differentiation-associated gene 5 antibodies in central Japan

We reviewed medical charts and examined the presence of anti-MDA-5 antibodies in 95 patients, including 36 CADM patients. Sera were obtained from 1994 through 2011. Statistical analyses were performed to assess whether CADM development and the presence of anti-MDA-5 antibodies were associated with various parameters, including age at disease onset, season of onset, annual positivity, and population of resident city.Tertiles based on the year when the sera were collected showed increasing tendencies of CADM and anti-MDA-5-positive patients among all of the dermatomyositis patients. From 1994 to 2010, the relative prevalence of CADM and anti-MDA-5 antibody-positive patients significantly increased. Interestingly, the presence of anti-MDA-5 antibodies in 26 patients was inversely associated with the population of their city of residence.This is the first study to examine the distribution of anti-MDA-5-positive dermatomyositis phenotypes in Japan. Regional differences in the incidences of these phenotypes would suggest that environmental factors contribute to the production of antibodies against MDA-5, which triggers innate antiviral responses.Idiopathic inflammatory myopathies are a heterogeneous group of autoimmune disorders that target the skeletal muscle and skin. Disease-related death is generally associated with malignancy and interstitial lung disease. The most frequent forms, polymyositis and dermatomyositis (DM), are thought to result from environmental exposure that leads to immune activation in genetically susceptible individuals. Several reports have found the onset or activity of inflammatory myopathies to show spatial clustering and seasonal association [1-5].A subgroup of DM patients who have typical skin manifestations of DM but little evidence of myositis has been recognized as clinically amyopathic dermatomyositis (CADM) [6]. Although it is still undetermined whether CADM is a distinct clinical entity or just an early phase of classic DM, rapidly progr