儿童型肌炎/皮肌炎临床和病理研究

Clinical and pathological characteristics of juvenile polymyositis/ dermatomyositis

摘要:

目的 儿童型多发性肌炎 /皮肌炎 (JPM /DM)目前在国内外文献中的大宗病例报道不多。为深入探讨两病的特征 ,本文着重研究其临床及病理特点 ,以进一步认识两病。方法 回顾性分析 4 2例JPM/DM (2 4例JPM ,1 8例JDM )患儿的临床资料 ,总结其临床表现、血清肌酶学、肌电图、肌肉病理的特点。结果 该病临床上主要有肌无力、肌萎缩 ,CK等血清肌酶增高 ,肌电图主要呈肌源性损害。JDM大多还有典型的皮肤损害 ,部分病例可出现皮下钙质沉积。JPM/DM的肌肉病理均显示免疫炎性改变。JPM组肌内膜炎发生率较JDM组高 (70 .8%vs 38.9% ,P <0 .0 5 ) ,JDM组肌束周萎缩及血管病变的发生率较JPM组高 (72 .2 %vs 4 1 .7% ,94 .4 %vs 5 4 .2 % ,P <0 .0 5或 0 .0 1 )。结论 JPM和JDM的临床和病理有所不同 ,JPM的病理改变可表现为散在肌纤维变性坏死、肌内膜炎改变为主的特征 ,部分病例微血管病变较明显 ;而JDM组的典型病理为广泛微血管病变和肌束周萎缩

Abstract:

Large series of case reports of juvenile polymyositis/dermatomyositis (JPM/JDM) are now seldom found in either Chinese or overseas literature. Clinical and pathological data of 42 cases of JPM/DM, including serum enzymes, electromyography (EMG) and muscular pathology were studied retrospectively. Patients with JPM/JDM manifested muscle weakness, muscle atrophy, elevation of creatine kinase and abnormal EMG with myogenic damage. Children with JDM generally manifested characteristic cutaneous abnormalities. Subcutaneous calcifications were present in some chronic cases of JDM. Muscle biopsy demonstrated immune inflammation in both JPM and JDM cases. The incidence of endomysium inflammatory in patients with JPM was higher than that in patients with JDM ( 70.8% vs 38.9% ) (P< 0.05 ), whereas the incidences of microvasculopathy ( 54.2% vs 94.4% ; P< 0.01 ) and perifascicular atrophy ( 41.7% vs 72.2% ; P< 0.05 ) was lower in JPM compared to JDM. Infiltrates were more frequently seen in JPM. Corticosteroid therapy was effective in most JPM/DM patients. It was concluded that there are some clinical and pathological differences between JPM and JDM. JPM frequently manifests sporadic muscle fibre degeneration, necrosis and endomysium inflammatory, while microvasculopathy and perifascicular atrophy are the characteristic pathology of JDM.