背景:幼年型皮肌炎仍然作為一個罕見的但最常見的兒童的發炎性肌肉疾病。目前沒有大規模的研究已經做了有關的幼年型皮肌炎的長期共病。 目的與方法:本研究使用全民健康保險研究資料庫來計算不同的2001年至2010的幼年性皮肌炎發病率和相關共病的危險比。 結果:2001年至2010共168新診斷幼年性皮肌炎患者。總體而言, 幼年性皮肌炎的平均發病率是3.12每百萬兒童,提供了一個男性對女性的比例為1:1.85 。幾乎所有年齡組中,女性發病率比男性高。長期共病的風險比顯著增加有感染疾病( HR : 3.118 , 95 % CI : 2.003 ? 4.854 ,P = <0.0001 ) ,肺結核病( HR : 10.057 , 95 % CI : 2.515 ? 40.215 ,P = 0.0011 )心血管疾病( HR : 4.986 ,95%CI : 2.643 ? 9.407 ,P = <0.0001 )和眼部疾病( HR : 9.655 ,95%CI : 3.801 ? 24.529 ,P = 0.0001 ) 。在感染疾病中,顯著增加危險比為細菌性鼻竇炎,尿路感染,腹腔膿腫/腹膜炎。 168病人中並沒有得惡性腫瘤共病,在此族群研究中。 結論: 台灣幼年性皮肌炎發病率與之前與英國,美國和日本的報告結果相似。長期共病有顯著感染,肺結核,心血管疾病和眼部疾病。惡性腫瘤並非長期共病。未來實施標準化疾病監測後,以幫助醫生確定被低估幼年皮肌炎的共病上實際的疾病風險,可能是重要的 Background:Juvenile dermatomyositis (JDM) remained as a rare but most common auto inflammatory myopathy in children. No large scale study had been done regarding long term comorbidities of the disease. Objective and Methods:This study used the National Health Insurance Research Database was used to calculate the incidence and hazard ratio of different comorbidities of JDM patients from 2001 to 2010. Results:A total of 168 newly diagnosis JDM patients were included in this cohort study from 2001 to 2010. Overall, the mean incidence of JDM was 3.12 per million children with a male to female ratio of 1:1.85. Female had a high incidence rate than male in nearly all age groups. Significant increase in hazard ratio (HR) were noted in infection (HR: 3.118, 95% CI: 2.003~4.854, P = <.0001), tuberculosis (HR: 10.057, 95% CI: 2.515~40.215, P = 0.0011), cardiovascular (HR: 4.986, 95% CI: 2.643~9.407, P = <.0001) and ocular diseases (HR: 9.655, 95% CI: 3.801~24.529, P = <.0001). Within infection group, significant increase in hazard ratio were bacterial sinusitis, urinary tract infection and abdominal abscess/peritonitis. None of the 168 JDM patient developed malignancy during this cohort study. Conclusion:Taiwan JDM incidence rate is in general comparable with previous reports from UK, US and Japan with a female predominance. Long term comorbidities consisted of infection, tuberculosis, cardiovascular and ocular diseases. Malignancy is not one of the comorbidities. Implementing standardized monitoring protocol may be vital to help determine the actual disease risk in some of the underestimated JDM comorbidities in future studies.