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幼年特发性皮肌炎:发病时 MRI 与临床结局的相关性。

Juvenile dermatomyositis: correlation of MRI at presentation with clinical outcome.

机构信息

Department of Pediatric Radiology, Cincinnati Children's Hospital Medical Center, OH 45229, USA.

出版信息

AJR Am J Roentgenol. 2011 Jul;197(1):W153-8. doi: 10.2214/AJR.10.5337.

DOI:10.2214/AJR.10.5337
PMID:21700978
Abstract

OBJECTIVE

The clinical course of juvenile dermatomyositis (JDMS) is unpredictable. MRI is used to determine muscle biopsy site and to monitor disease activity. It is unknown whether soft-tissue features on MRI obtained at diagnosis correlate with clinical outcome. The purpose of our study is to determine whether initial MRI findings in the pelvis and thighs in children with JDMS can predict clinical disease course.

MATERIALS AND METHODS

Forty-five children (31 girls and 14 boys; median age, 6 years; range, 1-18 years) with clinically diagnosed biopsy-proven JDMS and at least 24 months of clinical follow-up were included. Clinical outcome was categorized as limited or chronic disease, according to the established Crowe clinical classification scheme. Pretreatment MRI examinations of the pelvis and thighs were evaluated for signal abnormalities of muscle and fascia and reticulated signal changes in subcutaneous fat; associations with clinical outcome were examined.

RESULTS

Twenty-two patients had limited disease and 23 had chronic disease. Signal intensity ranged from normal (n = 3) to floridly increased in all muscle compartments (n = 17). Muscle and fascial involvement were not associated with clinical outcome. Controlling for duration of symptoms, the adjusted odds of progressing to chronic disease were higher for patients with abnormal subcutaneous fat signal than for those with normal fat signal (odds ratio, 9.0; 95% CI, 1.5-53.5; p < 0.02).

CONCLUSION

MRI findings of muscle or fascia involvement do not predict clinical outcome in children with newly diagnosed JDMS. Abnormal subcutaneous fat signal appears to have a significant association with a more aggressive chronic disease course.

摘要

目的

幼年皮肌炎(JDMS)的临床病程不可预测。MRI 用于确定肌肉活检部位并监测疾病活动度。尚不清楚在诊断时获得的 MRI 软组织特征是否与临床结局相关。我们的研究目的是确定 JDMS 患儿的骨盆和大腿的初始 MRI 发现是否可以预测临床疾病过程。

材料和方法

共纳入 45 例(31 名女孩和 14 名男孩;中位年龄为 6 岁;范围 1-18 岁)经临床诊断活检证实且至少有 24 个月临床随访的 JDMS 患儿。根据既定的 Crowe 临床分类方案,将临床结局分为局限性或慢性疾病。评估骨盆和大腿的预处理 MRI 检查,以评估肌肉和筋膜的信号异常以及皮下脂肪的网状信号变化;检查与临床结局的关联。

结果

22 例患者为局限性疾病,23 例为慢性疾病。信号强度从所有肌肉间隙正常(n = 3)到明显增加(n = 17)不等。肌肉和筋膜受累与临床结局无关。控制症状持续时间后,与脂肪信号正常的患者相比,脂肪信号异常的患者进展为慢性疾病的调整比值比更高(比值比,9.0;95%CI,1.5-53.5;p < 0.02)。

结论

新发 JDMS 患儿的 MRI 肌肉或筋膜受累表现并不能预测临床结局。异常的皮下脂肪信号似乎与更具侵袭性的慢性疾病过程有显著关联。

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