McCann L J, Garay S M, Ryan M M, Harris R, Riley P, Pilkington C A
Royal Liverpool NHS Trust, Alder Hey, Liverpool, UK.
Rheumatology (Oxford). 2007 Aug;46(8):1363-6. doi: 10.1093/rheumatology/kem131. Epub 2007 Jun 14.
To determine if objective, validated scores of muscle weakness and function [manual muscle testing (MMT), childhood myositis assessment scale (CMAS)] or scores of general disease activity or function [childhood health assessment questionnaire and physician global assessment of disease activity visual analogue scale (VAS)], can predict children at risk of swallow abnormalities in juvenile dermatomyositis (JDM) measured by videofluoroscopic swallow studies (VFSS).
Patients were referred for speech and language dysphagia assessment upon diagnosis of JDM or flare of disease. VFSS was used to document a swallow score indicating severity of swallow dysfunction. Clinical symptoms, examination findings and objective scores of disease activity were analysed. Any correlation was looked for using chi-squared Fisher exact test and linear regression models.
Fourteen patients with inflammatory myopathy (age 2-16 years) had clinical assessments and VFSS. VFSS was abnormal in 11 children (79%). Only two children were asymptomatic at assessment, but both had swallow dysfunction, including aspiration, on VFSS. In contrast, three of the symptomatic children had a normal VFSS. No relationship was found between objective disease severity scores and VFSS swallow score.
This study failed to show any correlation between swallow score and objective measures of muscle strength and function (MMT/CMAS) or general disease activity and function [physician VAS/childhood health assessment questionnaire (CHAQ)]. In the absence of a more accurate assessment method to determine which children with active JDM are most at risk of swallow dysfunction and aspiration, all children with active dermatomyositis should be referred for speech and language assessment and VFSS.
确定肌肉无力和功能的客观有效评分[徒手肌力测试（MMT）、儿童肌炎评估量表（CMAS）]或一般疾病活动或功能评分[儿童健康评估问卷和医生对疾病活动视觉模拟量表（VAS）评分]，能否预测通过电视荧光吞咽造影检查（VFSS）测量的青少年皮肌炎（JDM）吞咽异常风险的儿童。
患者在诊断为JDM或疾病发作时被转诊进行言语和语言吞咽障碍评估。VFSS用于记录吞咽评分，以表明吞咽功能障碍的严重程度。分析临床症状、检查结果和疾病活动的客观评分。使用卡方检验、费舍尔精确检验和线性回归模型寻找相关性。
14例炎性肌病患者（年龄2 - 16岁）进行了临床评估和VFSS检查。11名儿童（79%）的VFSS异常。评估时只有2名儿童无症状，但两人在VFSS上均有吞咽功能障碍，包括误吸。相比之下，3名有症状的儿童VFSS正常。未发现客观疾病严重程度评分与VFSS吞咽评分之间存在关联。
本研究未能显示吞咽评分与肌肉力量和功能的客观测量指标（MMT/CMAS）或一般疾病活动和功能[医生VAS/儿童健康评估问卷（CHAQ）]之间存在任何相关性。在缺乏更准确评估方法来确定哪些活动性JDM儿童吞咽功能障碍和误吸风险最高的情况下，所有活动性皮肌炎儿童均应转诊进行言语和语言评估及VFSS检查。
