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局限性硬皮病:硬斑病的解剖学磁共振成像表现及阅片者间分析

Localized scleroderma: anatomic MRI findings of morphea with inter-reader analysis.

作者信息

Iancau Alex, Attia Sarah, Duarte Silva Flavio, Eajazi Alireza, Malja Elona, Xi Yin, Jacobe Heidi, Chhabra Avneesh

机构信息

Medical School, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Department of Radiology, Musculoskeletal Division, University of Texas Southwestern Medical Center, Dallas, TX, USA.

出版信息

Skeletal Radiol. 2025 Mar 25. doi: 10.1007/s00256-025-04909-2.

Abstract

OBJECTIVE

To characterize the musculoskeletal lesions in patients with localized scleroderma (morphea) using MRI and examine inter-reader reliability.

MATERIALS AND METHODS

This was a retrospective study at a tertiary center with approval from the institutional review board. Forty-nine consecutive patients with final clinical diagnosis of Morphea (37 females, 12 males, mean age 39.04 ± 16.12 years) with different subtypes (20 Linear, 13 Generalized, 5 En coupe de Sabre, 4 Parry Romberg, 3 Eosinophilic Fasciitis 2 Plaque, and 2 Other) underwent MR imaging from 2016-2024. Three musculoskeletal radiologists evaluated different compartment involvements by Morphea- skin, fat, superficial/deep/intermuscular fascia, muscle/nerve, joint/bone, and depth of involvement as superficial or deep. Inter-reader analysis was performed using weighted Conger's Kappa and Intraclass Correlation Coefficient.

RESULTS

Among 49 patients with 49 MRI scans, 31 (63%) exhibited superficial lesions, and 18 (37%) had deep lesions. The MRI findings included- skin thickening in 37 (76%) and thinning in 10 (20%); fat atrophy in 35 (71%), and hypertrophy in 1 (2%). Thickening and edema were observed in 34 (69%) superficial fascia and 33 (67%) deep fascia. Muscle edema and bone/joint involvement were present in 11/18 deep lesions (61%) and 2 patients (4%), respectively. The depth of involvement showed good inter-rater agreement (ICC = 0.71 (95% CI: 0.58, 0.82)), with the highest reliability found for intermuscular fascial involvement (Conger's Kappa = 0.79).

CONCLUSION

We demonstrated an efficient MRI protocol for categorizing Morphea variations across tissue compartments, achieving good-fair inter-rater reliability, and identified deep tissue involvement in about one-third of cases.

摘要

目的

使用磁共振成像(MRI)对局限性硬皮病(硬斑病)患者的肌肉骨骼病变进行特征描述,并检验阅片者间的可靠性。

材料与方法

这是一项在三级医疗中心开展的回顾性研究,已获得机构审查委员会的批准。连续纳入49例最终临床诊断为硬斑病的患者(37例女性,12例男性,平均年龄39.04±16.12岁),其具有不同亚型(20例线性型、13例泛发型、5例剑砍状、4例帕里 - 罗姆伯格型、3例嗜酸性筋膜炎、2例斑块型和2例其他型),于2016年至2024年接受了MR成像检查。三名肌肉骨骼放射科医生评估硬斑病累及的不同部位——皮肤、脂肪、浅/深/肌间筋膜、肌肉/神经、关节/骨骼,以及累及深度为浅表或深部。采用加权康格卡方检验和组内相关系数进行阅片者间分析。

结果

在49例患者的49次MRI扫描中,31例(63%)表现为浅表病变,18例(37%)有深部病变。MRI表现包括——37例(7%)皮肤增厚,10例(20%)皮肤变薄;35例(71%)脂肪萎缩,1例(2%)脂肪肥大。34例(69%)浅筋膜和33例(67%)深筋膜出现增厚和水肿。11/18例深部病变(61%)出现肌肉水肿,2例患者(4%)出现骨/关节受累。累及深度显示出良好的阅片者间一致性(组内相关系数ICC = 0.71(95%可信区间:0.58,0.82)),肌间筋膜受累的可靠性最高(康格卡方系数 = 0.79)。

结论

我们展示了一种有效的MRI方案,可对硬斑病在不同组织部位的变化进行分类,阅片者间可靠性良好至中等,并在约三分之一的病例中发现了深部组织受累情况。

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