幼年皮肌炎诊断与治疗专家共识

Experts consensus on diagnosis and treatment of juvenile dermatomyositis

幼年皮肌炎(JDM)是儿童时期最常见的特发性炎症性肌病, 主要临床表现为特征性皮疹、四肢对称性近端肌无力和肌酶升高, 也可累及肺部、消化道等重要脏器。肌炎特异性抗体已成为JDM的特异性生物标志物, 不同的临床亚型具有不同的临床表现、疾病进程、治疗反应及远期预后。对JDM病情的评估及治疗应答情况的评价尤为重要。JDM的治疗多采用联合治疗方案, 包括糖皮质激素、免疫抑制剂、生物制剂或JAK抑制剂等小分子靶向药。本病异质性较大, 急性期如病情控制不佳易导致肌肉萎缩、肌腱挛缩和钙质沉着等远期后遗症。

Juvenile dermatomyositis(JDM)is the most common idiopathic inflammatory myopathy in children.Its main clinical manifestations include characteristic rash,symmetrical proximal muscle weakness and elevation of serum kinase,and the lungs,digestive tract and other important organs are also involved.Myositis specific antibody has become a specific biomarker of JDM.Clinical manifestations,disease progression,treatment response and long-term prognosis are different according to clinical subtypes.It is particularly important to evaluate the condition of the disease and the response to treatment.JDM is usually treated by combination of glucocorticoids,immunosuppressive agents,biologic agents or JAK inhibitors.The heterogeneity of this disease is large.If it is not well controlled in acute stage,long-term sequelae such as muscular atrophy,tendon contracture and calcinosis may occur.