Rosa Neto Nilton Salles, Goldenstein-Schainberg Cláudia
Medical School, Universidade de São Paulo (HCFMUSP), Brazil.
Rev Bras Reumatol. 2010 May-Jun;50(3):299-312.
Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by systemic vasculopathy. Its main manifestations include symmetrical proximal muscle weakness, elevated serum muscle enzymes and cutaneous lesions, among which the heliotrope and Gottron's papules are pathognomonic. Early recognition and prompt therapy allow better prognosis and prevent the development of calcinosis. Although the treatment is based on glucocorticoids, the more commonly associated immunosuppressors include methotrexate, azathioprine, cyclosporine, and cyclophosphamide, depending on the severity of disease. The use of immunobiologicals for refractory cases remains under investigation, but the results are controversial or inexpressive. In this review, we highlight recent updates on the pathogenesis and treatment of JDM.
幼年皮肌炎（JDM）是一种以系统性血管病变为特征的自身免疫性疾病。其主要表现包括对称性近端肌无力、血清肌酶升高和皮肤病变，其中向阳性皮疹和Gottron丘疹具有诊断意义。早期识别和及时治疗可获得更好的预后，并预防钙质沉着的发生。虽然治疗以糖皮质激素为基础，但根据疾病严重程度，更常用的联合免疫抑制剂包括甲氨蝶呤、硫唑嘌呤、环孢素和环磷酰胺。免疫生物制剂在难治性病例中的应用仍在研究中，但结果存在争议或无明显效果。在本综述中，我们重点介绍了JDM发病机制和治疗方面的最新进展。
