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Introduction of a novel magnetic resonance imaging-based scoring system for assessing disease activity in children

Mandela Thyoka1, Oba Adekunle, Clarissa Pilkington2

  • 1Department of Radiology, Sheffield Teaching Hospitals, Sheffield, UK.

Rheumatology (Oxford, England)
|June 15, 2018
PubMed

Insights

The juvenile dermatomyositis magnetic resonance Imaging Score (JIS) demonstrates good reliability for assessing disease severity in children. This MRI-based scoring system shows potential as a biomarker for treatment response in JDM.

Area of Science:

  • Pediatric Rheumatology
  • Radiology
  • Medical Imaging

Background:

  • Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
  • Accurate assessment of disease severity is crucial for effective management and monitoring treatment response in JDM.
  • Current methods for assessing JDM severity may lack objective measures, highlighting the need for reliable tools.

Purpose of the Study:

  • To develop and evaluate the reliability of a novel MRI-based scoring system, the juvenile dermatomyositis magnetic resonance Imaging Score (JIS).
  • To assess the inter- and intraobserver agreement of the JIS in scoring MRI findings in children with suspected JDM.

Main Methods:

  • Nine pediatric radiologists independently scored thigh MR images (axial and coronal views) from children with suspected JDM on two separate occasions.
  • The juvenile dermatomyositis magnetic resonance Imaging Score (JIS) was applied, with scores ranging from 0 (normal) to 100 (severe).
  • Inter- and intraobserver reliability were calculated using intraclass correlation coefficients (ICC) and Bland-Altman plots.

Main Results:

  • Good interobserver reliability was observed for both axial (ICC 0.87-0.88) and coronal (ICC 0.90) images.
  • Excellent intraobserver reliability was demonstrated, with ICC values ranging from 0.90 to 0.94.
  • The JIS showed consistent scoring across different readers and over time, indicating its robustness.

Conclusions:

  • The juvenile dermatomyositis magnetic resonance Imaging Score (JIS) is a reliable tool for assessing MRI findings in children with JDM.
  • JIS has the potential to serve as a semi-objective biomarker for disease severity and monitoring therapeutic interventions in JDM.
  • This scoring system can aid in standardizing the evaluation of JDM and improve patient care.
Abstract

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