骨骼肌单器官血管炎下肢肌肉磁共振成像特征及分型  

Characteristic manifestation of muscle pathology and the lower extremity magnetic resonance imaging and classification in patients with single-organ skeletal muscle vasculitis

作　　者：李嗣钊[1] 王玉丽[1] 卢昕[1] LI Si-zhao;WANG Yu-li;LU Xin(Department of Rheumatology,Key Laboratory of Myositis,China-Japan Friendship Hospital,Beijing 100029,China)

机构地区：[1]中日友好医院风湿免疫科,北京100029

出　　处：《中日友好医院学报》2024年第3期131-134,157,F0002,共6页Journal of China-Japan Friendship Hospital

摘　　要：目的:探究骨骼肌单器官血管炎(SoSMV)患者下肢肌肉磁共振成像(MRI)特征和临床分型。方法:回顾分析中日友好医院炎性肌病研究室/肌炎重点实验室2017年1月—2022年12月肌肉病理确诊的SoSMV患者的肌肉病理以及下肢肌肉MRI,包括治疗前的T1加权像(T1WI)、T2加权脂肪抑制像(T2WI-fs)和钆增强T1加权脂肪抑制像(Gd-T1WI-fs)。结果:共纳入SoSMV患者13例,其中男7例,女6例,中位发病年龄49岁。根据患者下肢肌肉MRI和肌肉病理特征,将患者分为2个亚型。1型SoSMV(n=6)患者下肢肌肉MRI均表现为受累肌肉明显肿胀,T1WI上表现为低信号伴有肌间脂肪信号数量减少,T2WI-fs上表现为弥漫分布的高信号,Gd-T1WI-fs上2例表现为比较均匀的强化;肌肉病理4例表现为坏死性血管炎,5例出现管腔闭塞,5例肌纤维变性、坏死伴炎细胞浸润和纤维脂肪组织增生。2型SoSMV(n=7),肌肉MRI均表现为受累肌肉无明显肿胀,T2WI上肌肉内可见边界不清的分枝状条纹高信号,即“鱼骨征”,相应部位在T1WI上的信号无异常,在Gd-T1WI-fs上3例表现为轻度强化;肌肉病理7例均表现为非坏死性血管炎伴血管周围炎。2型SoSMV患者的持续缓解率高于1型。结论:SoSMV患者下肢肌肉MRI和肌肉病理具有特征性表现,在SoSMV的诊断、分型和预后判断上为潜力指标。Objective:To describe the characteristic muscle pathology and lower extremity magnetic resonance imaging(MRI)findings of lower extremity muscles in patients with histopathological proven single-organ skeletal muscle vasculitis(SoSMV).Methods:The lower extremity MRI scans and muscle histological slides of patients with SoSMV diagnosis over 6 years were retrospectively evaluated.T1-weighted(T1WI)and T2-weighted fat-suppressed images(T2WI-fs)were available for all patients;additionally,in some cases gadolinium-enhanced T1-weighted fat-suppressed images(Gd-T1WI-fs)were also obtained.Results:A number of 13 patients were examined(7 males;median age[interquartile range]49 years[25-70]).Two distinct types of SoSMV were observed on MRI and pathology.For type-1 SoSMV(n=6),necrotizing vasculitis was detected in 4 patients and vessels with occluded lumen were identified in 5.Myofiber degeneration and necrosis,accompanied with inflammation and fibrosis were features in 5 and extensive in 3 of them.The affected muscles demonstrated hypointensively swelling with loss of the interspersed intramuscular fat on T1WI,diffuse hyperintense signal on T2WI-fs,and homogeneous enhancement on Gd-T1W-fs images(n=2).For type-2 SoSMV(n=7),perivasculitis and non-necrotizing vasculitis without occluded lumen were documented in all.The involved muscles revealed poorly defined,striated,and ramified hyperintense signals on axial T2WI-fs images,with the presence of a distinctive presentation,named as“fishbone sign”.The same areas were normal appearance on T1WI images and were slightly enhanced on Gd-T1WI-fs images(n=3).Conclusion:Lower extremity MRI scans showed a pattern of characteristic findings that was distinctive from different disease types,and might be useful non-invasively for assisting with the diagnosis,classification and outcome prediction of SoSMV.

关 键 词：血管炎 骨骼肌血管炎 单器官血管炎 磁共振成像 肌肉病理 

分 类 号：R543[医药卫生—心血管疾病]