Oropharyngeal dysphagia in juvenile dermatomyositis (JDM): an evaluation of videofluoroscopy swallow study (VFSS) changes in relation to clinical symptoms and objective muscle scores

Abstract

Objective: 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).

Methods: 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.

Results: 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.

Conclusions: 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.