Bowyer S L, Blane C E, Sullivan D B, Cassidy J T
J Pediatr. 1983 Dec;103(6):882-8. doi: 10.1016/s0022-3476(83)80706-9.
The medical records of 47 children with dermatomyositis who were seen in the pediatric rheumatology clinic at the University of Michigan between 1964 and 1982 were reviewed. Although most children with dermatomyositis have a good prognosis, the best predictor of both good functional recovery and minimal calcinosis is early treatment after the onset of symptoms, using high doses of prednisone for an adequate length of time. Of the children given such treatment, 78% had good functional outcomes, and disabling calcinosis was seen in 20% or less. Children given treatment late in the course of disease and with low doses of steroids are more likely to be functionally limited and have a greater amount of dystrophic calcium salt deposition. In our study, only 33% of patients given such treatment had a mild disease course with good functional outcome. We have identified a subgroup of children with dermatomyositis who appear to do poorly despite optimal therapeutic regimens. These patients are distinguished by a severe disease course responding minimally to corticosteroid therapy and manifested by persistent muscle weakness, elevations of muscle enzyme activity, and severe generalized cutaneous vasculitis. These children are at high risk for the development of exoskeleton-like calcification; consideration should be given to combined immunosuppressive therapy early in the course of disease.
对1964年至1982年间在密歇根大学儿科风湿病诊所就诊的47例皮肌炎患儿的病历进行了回顾。虽然大多数皮肌炎患儿预后良好，但症状出现后早期治疗，使用高剂量泼尼松并持续足够长的时间，是功能恢复良好和钙化最少的最佳预测因素。接受这种治疗的患儿中，78%功能结局良好，致残性钙化发生率在20%或更低。在疾病后期接受治疗且使用低剂量类固醇的患儿功能更易受限，营养不良性钙盐沉积量更大。在我们的研究中，接受这种治疗的患者中只有33%病程较轻且功能结局良好。我们已经确定了一组皮肌炎患儿，尽管采用了最佳治疗方案，但其病情似乎仍较差。这些患者的特点是病程严重，对皮质类固醇治疗反应极小，表现为持续性肌无力、肌肉酶活性升高和严重的全身性皮肤血管炎。这些患儿发生外骨骼样钙化的风险很高；在疾病早期应考虑联合免疫抑制治疗。
