PT - JOURNAL ARTICLE AU - Masatoshi Jinnin AU - Akiko Ohta AU - Shoichiro Ishihara AU - Hirofumi Amano AU - Tatsuya Atsumi AU - Manabu Fujimoto AU - Takashi Kanda AU - Yasushi Kawaguchi AU - Atsushi Kawakami AU - Akio Mimori AU - Tsuneyo Mimori AU - Toshihide Mimura AU - Yoshinao Muro AU - Hajime Sano AU - Jun Shimizu AU - Tsutomu Takeuchi AU - Yoshiya Tanaka AU - Kazuhiko Yamamoto AU - Takayuki Sumida AU - Hitoshi Kohsaka ED - , TI - First external validation of sensitivity and specificity of the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for idiopathic inflammatory myopathies with a Japanese cohort AID - 10.1136/annrheumdis-2019-215488 DP - 2020 Mar 01 TA - Annals of the Rheumatic Diseases PG - 387--392 VI - 79 IP - 3 4099 - http://ard.bmj.com/content/79/3/387.short 4100 - http://ard.bmj.com/content/79/3/387.full SO - Ann Rheum Dis2020 Mar 01; 79 AB - Objective To externally validate the performance of the new European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria set for idiopathic inflammatory myopathies (IIM) with a Japanese cohort.Methods This study included 420 IIM and 402 non-IIM cases. Probability of having IIM in each patient was calculated using the collected data set. The cut-off probability was set at 55%, as recommended by EULAR/ACR. Patients classified as IIM by the criteria were further subclassified with classification trees.Results When the probability cut-off was set at 55%, the sensitivity/specificity of the new criteria to diagnose IIM were 89.3%/91.0% in the total cohort, 88.1%/95.1% without muscle biopsy data and 90.4%/65.5% with biopsy data. The cohort included 12 overlap syndrome patients with biopsy data, who were included as non-IIM cases in accordance with traditional Japanese methods. When they were included in the IIM cases, the specificity in patients with biopsy increased to 74.4%. The sensitivity/specificity of the new criteria to diagnose polymyositis/dermatomyositis (PM/DM) plus juvenile and amyopathic DM in the Japanese cohort was 87.4%/92.4%, which were greater than those of the Tanimoto’s criteria revised to enable classification of amyopathic DM (ADM) (71.2%/87.8%) and were comparable with those of Bohan & Peter’s criteria to diagnose those diseases except for ADM (88.4%/88.3%).Conclusions Our study externally validated high specificity of the new criteria for the first time, although with several limitations, including low percentage of child patients. The new criteria have higher sensitivity and/or specificity in classification of PM/DM than the previously reported criteria, demonstrating its usefulness for interethnic patients.