Zheng Yiming, Liu Linlin, Wang Lu, Xiao Jiangxi, Wang Zhaoxia, Lv He, Zhang Wei, Yuan Yun
Department of Neurology and Department of Radiology, Peking University First Hospital, Peking, China.
Department of Neurology and Department of Radiology, Peking University First Hospital, Peking, China
Rheumatology (Oxford). 2015 Jun;54(6):1017-24. doi: 10.1093/rheumatology/keu422. Epub 2014 Nov 21.
The aim of this study was to evaluate muscle MRI changes and the role of MRI in monitoring therapy in patients with myopathy associated with antibodies to signal recognition particle (anti-SRP myopathy).
We identified 12 patients with anti-SRP myopathy [6 females and 6 males; mean age of onset 38.5 years (s.d. 12.4), mean duration 22.8 months (s.d. 20.6). The main symptoms were proximal limb muscle weakness. Mean serum creatine kinase levels were moderately increased. Muscle biopsies revealed necrotizing myopathy in all patients, with obvious connective tissue proliferation in five patients and a single focus of lymphocytic infiltration in the endomysium in one. The myositis disease activity assessment (MYOACT) visual analogue scales scores were assessed. Muscle MRI was performed through the thighs. All patients were treated with corticosteroids and other immunosuppressive drugs.
MRI revealed fatty infiltration and oedema in the thigh muscles of all 12 patients. Prominent fatty infiltration was present in 4 of the 12 patients. The hamstrings and adductor magnus were the most severely infiltrated and the quadriceps femoris the least. Obvious oedema was observed in 10 of the 12 patients, the most severely affected muscles being the vastus lateralis, rectus femoris, biceps femoris and adductor magnus, with relative sparing of the vastus intermedius. The degree of oedema was not correlated with creatine kinase levels or MYOACT scores. The four patients with striking fatty infiltration were refractory to therapy.
MRI of the thigh muscles shows a distinct pattern of oedema and fatty infiltration and can be used to monitor the treatment of patients with anti-SRP myopathy.
本研究旨在评估与信号识别颗粒抗体相关的肌病(抗SRP肌病)患者的肌肉MRI变化以及MRI在监测治疗中的作用。
我们纳入了12例抗SRP肌病患者[6例女性和6例男性;平均发病年龄38.5岁(标准差12.4),平均病程22.8个月(标准差20.6)。主要症状为近端肢体肌无力。平均血清肌酸激酶水平中度升高。肌肉活检显示所有患者均为坏死性肌病,5例患者有明显的结缔组织增生,1例患者在内膜有单个淋巴细胞浸润灶。评估了肌炎疾病活动评估(MYOACT)视觉模拟量表评分。对大腿进行了肌肉MRI检查。所有患者均接受了皮质类固醇和其他免疫抑制药物治疗。
MRI显示所有12例患者大腿肌肉均有脂肪浸润和水肿。12例患者中有4例存在明显的脂肪浸润。绳肌和大收肌浸润最严重,股四头肌最轻。12例患者中有10例观察到明显水肿,受影响最严重的肌肉为股外侧肌、股直肌、股二头肌和大收肌,股中间肌相对较轻。水肿程度与肌酸激酶水平或MYOACT评分无关。4例有明显脂肪浸润的患者对治疗无效。
大腿肌肉MRI显示出独特的水肿和脂肪浸润模式,可用于监测抗SRP肌病患者的治疗。