Ito S, Miyamae T, Imagawa T, Katakura S, Mori M, Tomono J, Ibe M, Mituda T, Aihara Y, Yokota S
Department of Pediatrics, Yokohama City University, School of Medicine.
Ryumachi. 1998 Dec;38(6):785-92.
Juvenile dermatomyositis (JDM) is characterized by microvasculopathy of the striated muscle, which indicates different etiology, clinical manifestation and prognosis from the adult-onset dermatomyositis. We experienced 10 cases of JDM and 1 case of juvenile polymyositis (JPM) in the recent 14 years, and analyzed clinical manifestation, laboratory findings, treatment anrognosis. The cases were 9 girls and 2 boys. The onset of the disease was 2 years of age in 2 patients, and 9 to 13 years of age in 9 patients. During the follow-up courses, no cases were dead or complicated with neoplasm. Skin rash was the most frequent manifestation at the onset, and facial erythema was common. Muscle weakness was observed only in 4 cases at the onset, and in all cases muscle enzymes including creatine kinase and aldolase were elevated. The clinical course was classified into three groups; monocyclic (5 cases), chronic and recurrent (4 cases), and fulminant (2 cases). Prognosis depended not on the degree of the elevated serum muscle enzymes, but on the initial therapy employed at the onset of the disease. Five cases including 2 cases of fulminant type were initially treated with methylprednisolon pulse therapy, and all of these had no recurrence. On the other hand, 6 cases were started the therapy with p.o. prednisolone. Four of them had frequent recurrences in accordance with tapering of prednisolone. These cases were effectively treated with the combination with immunosuppressants. In previous reports, JDM and JPM were reported to be a disorder which had relatively favorable prognosis. But we found that one third of the cases had chronic and recurrent courses. Methylprednisolone pulses as initial therapy may be effective in preventing the chronicity and recurrence of the disease.
幼年皮肌炎（JDM）的特征是横纹肌微血管病变，这表明其病因、临床表现和预后与成人皮肌炎不同。在最近14年中，我们诊治了10例JDM和1例幼年型多发性肌炎（JPM），并分析了其临床表现、实验室检查结果、治疗及预后。病例中9例为女孩，2例为男孩。2例患者发病年龄为2岁，9例患者发病年龄为9至13岁。在随访过程中，无病例死亡或并发肿瘤。皮疹是最常见的首发表现，面部红斑很常见。仅4例患者首发时有肌无力表现，所有病例的肌酸激酶和醛缩酶等肌酶均升高。临床病程分为三组：单循环型（5例）、慢性复发型（4例）和暴发型（2例）。预后不取决于血清肌酶升高的程度，而是取决于疾病发作时采用的初始治疗。包括2例暴发型在内的5例患者最初采用甲泼尼龙冲击疗法治疗，所有这些患者均无复发。另一方面，6例患者开始口服泼尼松龙治疗。其中4例随着泼尼松龙减量出现频繁复发。这些病例联合免疫抑制剂治疗有效。在以往的报道中，JDM和JPM被报道为预后相对较好的疾病。但我们发现三分之一的病例有慢性复发病程。甲泼尼龙冲击作为初始治疗可能有效预防疾病的慢性化和复发。
