皮肌炎（dermatomyositis,DM）和多发性肌炎（polymyositis,PM）是一类系统性自身免疫性疾病,主要累及皮肤、骨骼肌、肺等多个器官。其发病机制目前尚不明确,研究发现许多自身抗体与疾病的临床特征具有明显相关性,这些自身抗体包括肌炎...皮肌炎（dermatomyositis,DM）和多发性肌炎（polymyositis,PM）是一类系统性自身免疫性疾病,主要累及皮肤、骨骼肌、肺等多个器官。其发病机制目前尚不明确,研究发现许多自身抗体与疾病的临床特征具有明显相关性,这些自身抗体包括肌炎特异性抗体（myositis specific autoantibodies,MSAs）、肌炎相关性抗体（myositis associated autoantibodies,MAAs）以及组织特异性自身抗体。展开更多
Objective:To evaluate the impact of duration of untreated symptoms in childre n with juvenile dermatomyositis(JDM)on clinical and laboratory status at diagn osis.Study design:We examined physical and laboratory data f...Objective:To evaluate the impact of duration of untreated symptoms in childre n with juvenile dermatomyositis(JDM)on clinical and laboratory status at diagn osis.Study design:We examined physical and laboratory data from the first phys ician visit for 166 untreated children with JDM.Disease activity scores(DASs)assessed skin and muscle involvement.Height and weight were compared with the N ational Health and Nutrition Examination Survey III dataset.Duration of untreat ed illness was designated as the time from first sign of rash or weakness to dia gnostic visit.Results:Boys and girls with untreated JDM were shorter and light er than national norms(P >.0005 for both),and nonwhite children were weaker than wh ite children(P >.0005).Older children had more dysphagia(P =.017)and arthr itis(P >.001).Duration of untreated JDM was negatively associated with DAS we akness(P >.0005),unrelated to DAS skin,and positively associated with pathol ogical calcifications(P =.006).With untreated disease < 4.7 months,serum lev els of 4 muscle enzymes(aldolase,lactic dehydrogenase,creatine kinase,serum glutamic-oxaloacetic transaminase/aspartate aminotransferase)tended toward nor mal(P >.01 for each).Conclusions:Duration of untreated symptoms is an import ant variable and should be included in decisions concerning both diagnostic crit eria and intensity of therapy for children with JDM.展开更多
Juvenile dermatomyositis(JDM)is a chronic pediatric autoimmune disease characterized by proximal muscle weakness and distinctive skin manifestations[1,2].The first-line treatment for JDM involves the combination of gl...Juvenile dermatomyositis(JDM)is a chronic pediatric autoimmune disease characterized by proximal muscle weakness and distinctive skin manifestations[1,2].The first-line treatment for JDM involves the combination of glucocorticoids with either methotrexate(MTX)or cyclosporine[3,4].For severe and refractory juvenile dermatomyositis,options such as tacrolimus.展开更多
文摘皮肌炎（dermatomyositis,DM）和多发性肌炎（polymyositis,PM）是一类系统性自身免疫性疾病,主要累及皮肤、骨骼肌、肺等多个器官。其发病机制目前尚不明确,研究发现许多自身抗体与疾病的临床特征具有明显相关性,这些自身抗体包括肌炎特异性抗体（myositis specific autoantibodies,MSAs）、肌炎相关性抗体（myositis associated autoantibodies,MAAs）以及组织特异性自身抗体。
文摘Objective:To evaluate the impact of duration of untreated symptoms in childre n with juvenile dermatomyositis(JDM)on clinical and laboratory status at diagn osis.Study design:We examined physical and laboratory data from the first phys ician visit for 166 untreated children with JDM.Disease activity scores(DASs)assessed skin and muscle involvement.Height and weight were compared with the N ational Health and Nutrition Examination Survey III dataset.Duration of untreat ed illness was designated as the time from first sign of rash or weakness to dia gnostic visit.Results:Boys and girls with untreated JDM were shorter and light er than national norms(P >.0005 for both),and nonwhite children were weaker than wh ite children(P >.0005).Older children had more dysphagia(P =.017)and arthr itis(P >.001).Duration of untreated JDM was negatively associated with DAS we akness(P >.0005),unrelated to DAS skin,and positively associated with pathol ogical calcifications(P =.006).With untreated disease < 4.7 months,serum lev els of 4 muscle enzymes(aldolase,lactic dehydrogenase,creatine kinase,serum glutamic-oxaloacetic transaminase/aspartate aminotransferase)tended toward nor mal(P >.01 for each).Conclusions:Duration of untreated symptoms is an import ant variable and should be included in decisions concerning both diagnostic crit eria and intensity of therapy for children with JDM.
基金supported by the National Key R&D Program of China(Grant No.2021YFC2702001)Project supported by the Young Scientists Fund of the National Natural Science Foundation of China(Grant No.82101423).
文摘Juvenile dermatomyositis(JDM)is a chronic pediatric autoimmune disease characterized by proximal muscle weakness and distinctive skin manifestations[1,2].The first-line treatment for JDM involves the combination of glucocorticoids with either methotrexate(MTX)or cyclosporine[3,4].For severe and refractory juvenile dermatomyositis,options such as tacrolimus.
