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Patients with anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 autoantibodies were divided into 2 subgroups: those with and those without calcinosis. Associations of anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 autoantibody levels with organ\u2010specific disease activity (using 10\u2010cm visual analog scale [<jats:styled-content style=\"fixed-case\">VAS<\/jats:styled-content>] scores), serum creatine kinase (<jats:styled-content style=\"fixed-case\">CK<\/jats:styled-content>) levels, and calcinosis severity were investigated in cross\u2010sectional and longitudinal analyses.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A cross\u2010sectional analysis of 56 <jats:styled-content style=\"fixed-case\">IIM<\/jats:styled-content> patients with anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 autoantibodies (38 without calcinosis and 18 with calcinosis) showed that in patients without calcinosis, the levels of anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 autoantibodies were positively correlated with the physician's global assessment of disease activity and muscle <jats:styled-content style=\"fixed-case\">VAS<\/jats:styled-content> scores and serum <jats:styled-content style=\"fixed-case\">CK<\/jats:styled-content> levels, whereas no such association was found in patients with calcinosis. Results of the longitudinal study revealed strong correlations of anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 antibody levels with the physician's global assessment and constitutional, cutaneous, gastrointestinal, and muscle <jats:styled-content style=\"fixed-case\">VAS<\/jats:styled-content> scores and serum <jats:styled-content style=\"fixed-case\">CK<\/jats:styled-content> levels in patients without calcinosis, but in patients with calcinosis, only a moderate correlation was observed between anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 antibody levels and the physician's global <jats:styled-content style=\"fixed-case\">VAS<\/jats:styled-content> and constitutional <jats:styled-content style=\"fixed-case\">VAS<\/jats:styled-content> scores. Of note, in patients without calcinosis, anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 autoantibodies were found to disappear during periods of clinical remission, but reappeared with disease relapse. No association between anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 antibody levels and the severity of calcinosis was observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These findings indicate that anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 autoantibodies serve as a useful marker for disease activity in patients with <jats:styled-content style=\"fixed-case\">IIM<\/jats:styled-content>s, especially in the absence of calcinosis. The differential associations observed between anti\u2013<jats:styled-content style=\"fixed-case\">NXP<\/jats:styled-content>\u20102 autoantibody levels and disease activity suggest that there may be a phenotypic difference between patients with and those without calcinosis.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.40491","type":"journal-article","created":{"date-parts":[[2018,3,15]],"date-time":"2018-03-15T07:20:14Z","timestamp":1521098414000},"page":"1288-1297","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Differential Clinical Associations of Anti\u2013Nuclear Matrix Protein 2 Autoantibodies in Patients With Idiopathic Inflammatory Myopathies"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0665-4692","authenticated-orcid":false,"given":"Hanbo","family":"Yang","sequence":"first","affiliation":[{"name":"China\u2013Japan Friendship Hospital and Graduate School of Peking 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