Pachman L M, Hayford J R, Chung A, Daugherty C A, Pallansch M A, Fink C W, Gewanter H L, Jerath R, Lang B A, Sinacore J, Szer I S, Dyer A R, Hochberg M C
Division of Immunology, Children's Memorial Hospital/Northwestern University Medical School, Chicago, Illinois 60614, USA.
J Rheumatol. 1998 Jun;25(6):1198-204.
To evaluate demographic and clinical characteristics, duration of time between disease onset (date of first rash and/or weakness), and diagnosis/therapy, as well as socioeconomic status, of children with newly diagnosed juvenile dermatomyositis (JDM).
Structured telephone interview of families of a cohort of 79 children with JDM: interval between onset of symptoms to diagnosis, median of 3 months (range 0.5-20.0).
At diagnosis, all the children had rash (100%) and proximal muscle weakness (100%); 58 (73%) had muscle pain; 51 (65%) fever; 35 (44%) dysphagia; 34 (43%) hoarseness; 29 (37%) abdominal pain; 28 (35%) arthritis; 18 (23%) calcinosis, and 10 (13%) melena. Muscle derived enzymes were normal in 10% of the children. Of the 43 children who had an electromyogram (EMG), 8 (19%) had normal results. Fifty-one children had a muscle biopsy; the results were normal/nondiagnostic in 10 (20%). Median time from disease onset to diagnosis was different between racial groups: Caucasians (n=59) 2.0 months: for minorities (n=20), 6.5 months, (p=0.0008). The median time from disease onset to therapy was: Caucasians. 3.0 months; minorities, 7.2 months (p=0.002). Report of calcinosis was associated with increased time to diagnosis and therapy (p=0.04). In the 33 children whose first symptom occurred in June-September, rash preceded or accompanied onset of muscle weakness in 83% (n=27). Ninety-one percent of the children were given steroid therapy and 9% received methotrexate as well.
The results of an undirected site for muscle biopsy or EMG may not be diagnostic. Minority children had a longer interval between first JDM symptom and diagnosis/therapy than Caucasian children. Delay in diagnosis/therapy was associated with calcinosis.
评估新诊断的幼年皮肌炎（JDM）患儿的人口统计学和临床特征、疾病发作（首次出现皮疹和/或肌无力的日期）至诊断/治疗的时间间隔以及社会经济状况。
对79例JDM患儿的家庭进行结构化电话访谈：症状出现至诊断的间隔时间，中位数为3个月（范围0.5 - 20.0个月）。
诊断时，所有患儿均有皮疹（100%）和近端肌无力（100%）；58例（73%）有肌肉疼痛；51例（65%）发热；35例（44%）吞咽困难；34例（43%）声音嘶哑；29例（37%）腹痛；28例（35%）关节炎；18例（23%）钙质沉着，10例（13%）黑便。10%的患儿肌肉衍生酶正常。43例行肌电图（EMG）检查的患儿中，8例（19%）结果正常。51例患儿进行了肌肉活检；10例（20%）结果正常/无诊断意义。不同种族患儿从疾病发作至诊断的中位数时间不同：白种人（n = 59）为2.0个月；少数族裔（n = 20）为6.5个月，（p = 0.0008）。从疾病发作至治疗的中位数时间为：白种人3.0个月；少数族裔7.2个月（p = 0.002）。钙质沉着的报告与诊断和治疗时间延长相关（p = 0.04）。在33例首发症状出现在6 - 9月的患儿中，83%（n = 27）皮疹先于或伴随肌无力发作。91%的患儿接受了类固醇治疗，9%还接受了甲氨蝶呤治疗。
无针对性的肌肉活检或EMG检查结果可能无法确诊。少数族裔患儿从首次出现JDM症状至诊断/治疗的时间间隔比白种人患儿长。诊断/治疗延迟与钙质沉着相关。
