Pachman Lauren M, Abbott Kathy, Sinacore James M, Amoruso Lisa, Dyer Alan, Lipton Rebecca, Ilowite Norman, Hom Christine, Cawkwell Gail, White Andrew, Rivas-Chacon Rafael, Kimura Yukiko, Ray Linda, Ramsey-Goldman Rosalind
Department of Pediatrics, Division of Immunology/Rheumatology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA. pachman@#northwestern.edu
J Pediatr. 2006 Feb;148(2):247-53. doi: 10.1016/j.jpeds.2005.10.032.
To evaluate the impact of duration of untreated symptoms in children with juvenile dermatomyositis (JDM) on clinical and laboratory status at diagnosis.
We examined physical and laboratory data from the first physician visit for 166 untreated children with JDM. Disease activity scores (DASs) assessed skin and muscle involvement. Height and weight were compared with the National Health and Nutrition Examination Survey III dataset. Duration of untreated illness was designated as the time from first sign of rash or weakness to diagnostic visit.
Boys and girls with untreated JDM were shorter and lighter than national norms (P > .0005 for both), and nonwhite children were weaker than white children (P > .0005). Older children had more dysphagia (P = .017) and arthritis (P > .001). Duration of untreated JDM was negatively associated with DAS weakness (P > .0005), unrelated to DAS skin, and positively associated with pathological calcifications (P = .006). With untreated disease > or = 4.7 months, serum levels of 4 muscle enzymes (aldolase, lactic dehydrogenase, creatine kinase, serum glutamic-oxaloacetic transaminase/aspartate aminotransferase) tended toward normal (P > .01 for each).
Duration of untreated symptoms is an important variable and should be included in decisions concerning both diagnostic criteria and intensity of therapy for children with JDM.
评估幼年皮肌炎（JDM）患儿未治疗症状持续时间对诊断时临床和实验室状况的影响。
我们检查了166例未经治疗的JDM患儿首次就诊时的体格检查和实验室数据。疾病活动评分（DAS）评估皮肤和肌肉受累情况。将身高和体重与第三次全国健康和营养检查调查数据集进行比较。未治疗疾病的持续时间定义为从皮疹或肌无力的首次出现到诊断就诊的时间。
未经治疗的JDM患儿，男孩和女孩均比全国标准身高更矮、体重更轻（两者P>.0005），非白人儿童比白人儿童更虚弱（P>.0005）。年龄较大的儿童吞咽困难更多（P=.017），关节炎更多（P>.001）。未治疗的JDM持续时间与DAS肌无力呈负相关（P>.0005），与DAS皮肤无关，与病理性钙化呈正相关（P=.006）。未治疗疾病≥4.7个月时，4种肌肉酶（醛缩酶、乳酸脱氢酶、肌酸激酶、血清谷氨酸草酰乙酸转氨酶/天冬氨酸转氨酶）的血清水平趋于正常（每种酶P>.01）。
未治疗症状的持续时间是一个重要变量，在制定JDM患儿的诊断标准和治疗强度决策时应予以考虑。
