Abstract
Objectives
Juvenile localized scleroderma (JLS) is a rare chronic autoimmune disease which can also affect bones and muscles. Nevertheless, muscle loss was not previously investigated in patients with JLS. Thus, the aim of this study was to retrospectively evaluate deep involvement and assess and quantify sarcopenia in JLS patients using magnetic resonance imaging (MRI).
Methods
Fourteen children with JLS (nine females, mean age ± SD, 7.1 ± 3.6 years) referring to our tertiary center from January 2012 to January 2018 who underwent at least one MRI examination including axial T1-weighted and short tau inversion recovery images were included. Two readers assessed in consensus superficial and deep involvement. Muscle edema, muscle fatty infiltration, and sarcopenia were independently scored (absent, moderate, or severe) and the Cohen’s kappa coefficient computed. Skin perimeter, subcutaneous area, muscle area, and muscle volume were independently measured using the contralateral unaffected extremity as reference (paired Student’s t test, p < 0.05). The intraclass correlation coefficient (ICC) was used to investigate the reliability of the measurements.
Results
All patients showed superficial involvement with subcutaneous fat atrophy being the most common finding (13 patients). Bone marrow edema occurred in five patients. Muscle edema affected ten children (moderate in seven, severe in three; k = 0.89), muscle fatty replacement occurred in one case (severe; k = 1.00), and sarcopenia was detected in eight patients (severe in two; k = 0.78). All quantitative parameters were lower on the affected side than on the unaffected contralateral limb (p < 0.05, each) and all measurements showed a high reliability (ICC > 0.750, each).
Conclusion
Patients with JLS can be affected by sarcopenia and quantitative analyses allow a robust characterization of such finding.
Key Points
• Deep involvement in juvenile localized scleroderma is frequently characterized by sarcopenia.
• In juvenile localized scleroderma, muscle edema and sarcopenia are mostly moderate while fatty infiltration, even if rare, can be severe.
• Sarcopenia can be reliably quantified in children with juvenile localized scleroderma using MRI.
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Abbreviations
- CI:
-
Confidence interval
- ICC:
-
Intraclass correlation coefficient
- JLS:
-
Juvenile localized scleroderma
- k :
-
Cohen’s kappa
- MA:
-
Muscle area
- MRI:
-
Magnetic resonance imaging
- MV:
-
Muscle volume
- SA:
-
Subcutaneous area
- SP:
-
Skin perimeter
- STIR:
-
Short tau inversion recovery
- TSE:
-
Turbo spin echo
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The scientific guarantor of this publication is Dr. Chiara Giraudo, MD, PhD.
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Michael Weber kindly provided statistical advice for this manuscript.
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Written informed consent was not required for this study because it is retrospective but the parents gave written informed consent to the MRI examination, as usually performed in clinical practice, since all patients were minor.
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Institutional Review Board approval was obtained.
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• Retrospective
• Cross sectional study
• Performed at one institution
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Flores Quispe, S.K.J., Cavaliere, A., Weber, M. et al. Sarcopenia in juvenile localized scleroderma: new insights on deep involvement. Eur Radiol 30, 4091–4097 (2020). https://doi.org/10.1007/s00330-020-06764-2
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DOI: https://doi.org/10.1007/s00330-020-06764-2