Department of Medicine - DIMED, Radiology Institute, University of Padova, Via Giustiniani 2, 35100, Padua, Italy.
Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria.
Eur Radiol. 2020 Jul;30(7):4091-4097. doi: 10.1007/s00330-020-06764-2. Epub 2020 Mar 6.
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).
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.
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).
Patients with JLS can be affected by sarcopenia and quantitative analyses allow a robust characterization of such finding.
• 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.
幼年局限性硬皮病（JLS）是一种罕见的慢性自身免疫性疾病，也可能影响骨骼和肌肉。然而，以前并未研究过 JLS 患者的肌肉减少症。因此，本研究的目的是使用磁共振成像（MRI）回顾性评估深部受累情况，并评估和量化 JLS 患者的肌肉减少症。
2012 年 1 月至 2018 年 1 月期间，我们的三级中心收治了 14 例 JLS 患儿（9 名女性，平均年龄±标准差，7.1±3.6 岁），他们至少接受了一次 MRI 检查，包括轴向 T1 加权和短 tau 反转恢复图像。两名读者共同评估了浅层和深层受累情况。肌肉水肿、肌肉脂肪浸润和肌肉减少症分别进行评分（无、中度或重度），并计算 Cohen's kappa 系数。使用对侧未受影响的肢体作为参考（配对学生 t 检验，p<0.05）分别独立测量皮肤周长、皮下面积、肌肉面积和肌肉体积。使用组内相关系数（ICC）评估测量的可靠性。
所有患者均表现为浅层受累，最常见的发现是皮下脂肪萎缩（13 例）。五例患者出现骨髓水肿。10 例患儿出现肌肉水肿（7 例中度，3 例重度；k=0.89），1 例出现肌肉脂肪替代（重度；k=1.00），8 例出现肌肉减少症（2 例重度；k=0.78）。受累侧的所有定量参数均低于未受累的对侧肢体（p<0.05，每项），所有测量均具有较高的可靠性（ICC>0.750，每项）。
JLS 患者可能患有肌肉减少症，定量分析可对该发现进行可靠的描述。
• 幼年局限性硬皮病的深部受累常伴有肌肉减少症。• 在幼年局限性硬皮病中，肌肉水肿和肌肉减少症多为中度，而脂肪浸润虽然罕见，但可能为重度。• 使用 MRI 可以可靠地量化儿童的肌肉减少症。
