Faculdade de Medicina de Botucatu, UNESP, São Paulo, Brazil.
Clin Exp Rheumatol. 2009 Nov-Dec;27(6):1031-8.
To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry.
Inclusion criteria were onset age lower than 18 years and a diagnosis of any idiopathic inflammatory myopathy (IIM) by attending physician. Bohan & Peter (1975) criteria categorisation was established by a scoring algorithm to define JDM and JPM based on clinical protocol data.
Of the 189 cases included, 178 were classified as JDM, 9 as JPM (19.8: 1) and 2 did not fit the criteria; 6.9% had features of chronic arthritis and connective tissue disease overlap. Diagnosis classification agreement occurred in 66.1%. Median onset age was 7 years, median follow-up duration was 3.6 years. Malignancy was described in 2 (1.1%) cases. Muscle weakness occurred in 95.8%; heliotrope rash 83.5%; Gottron plaques 83.1%; 92% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 74.6% was abnormal in 91.5% and electromyogram performed in 39.2% resulted abnormal in 93.2%. Logistic regression analysis was done in 66 cases with all parameters assessed and only aldolase resulted significant, as independent variable for definite JDM (OR=5.4, 95%CI 1.2-24.4, p=0.03). Regarding treatment, 97.9% received steroids; 72% had in addition at least one: methotrexate (75.7%), hydroxychloroquine (64.7%), cyclosporine A (20.6%), IV immunoglobulin (20.6%), azathioprine (10.3%) or cyclophosphamide (9.6%). In this series 24.3% developed calcinosis and mortality rate was 4.2%.
Evaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with definite JDM category. In practice, prednisone-methotrexate combination was the most indicated treatment.
描述多中心登记处的青少年皮肌炎（JDM）和青少年多发性肌炎（JPM）的发病特征、分类和治疗。
纳入标准为发病年龄低于 18 岁，且主治医师诊断为任何特发性炎症性肌病（IIM）。根据临床方案数据，采用评分算法建立 Bohan & Peter（1975）标准分类，以定义 JDM 和 JPM。
在纳入的 189 例病例中，178 例被归类为 JDM，9 例为 JPM（19.8:1），2 例不符合标准；6.9%的患者具有慢性关节炎和结缔组织病重叠的特征。诊断分类的一致性为 66.1%。中位发病年龄为 7 岁，中位随访时间为 3.6 年。2 例（1.1%）患者有恶性肿瘤描述。95.8%的患者有肌肉无力；83.5%的患者有向阳疹；83.1%的患者有 Gottron 斑；92%的患者至少有一项异常的肌肉酶结果。74.6%的患者进行了肌肉活检，91.5%的肌肉活检异常；39.2%的患者进行了肌电图检查，93.2%的肌电图异常。对 66 例患者进行了所有参数的逻辑回归分析，仅发现醛缩酶有意义，是明确 JDM 的独立变量（OR=5.4，95%CI 1.2-24.4，p=0.03）。关于治疗，97.9%的患者接受了类固醇治疗；72%的患者除了类固醇之外还接受了至少一种药物治疗：甲氨蝶呤（75.7%）、羟氯喹（64.7%）、环孢素 A（20.6%）、静脉注射免疫球蛋白（20.6%）、硫唑嘌呤（10.3%）或环磷酰胺（9.6%）。在该系列中，24.3%的患者发生了钙质沉着症，死亡率为 4.2%。
评估明确诊断的预设标准表明，醛缩酶是与明确 JDM 分类最相关的最重要参数。实际上，泼尼松-甲氨蝶呤联合治疗是最适用的治疗方法。
