幼年皮肌炎20例临床特点及转归分析

Analysis of Clinical Characteristics and Prognosis in 20 Juvenile Dermatomyositis

目的探讨幼年皮肌炎(JDM)的临床特点、治疗措施和转归。方法回顾性分析2014年1月-2018年9月西安交通大学医学院附属儿童医院免疫科住院的20例JDM患儿的临床表现、实验室检查、治疗方案及预后。结果男8例,女12例,发病年龄1.10岁,平均发病年龄5.65岁。所有患儿均有典型的皮损及肌无力表现。16例患儿有Gottron丘疹,10例存在内脏受累,最常受累为呼吸系统(7例)。所有患儿均有不同程度血清肌酶增高,乳酸脱氢酶(LDH)增高18例,肌酸激酶(CK)增高13例。肌电图均表现为肌源性损害,其中14例行肌肉活检者病理结果均符合皮肌炎改变。18例患儿接受糖皮质激素治疗,其中2例给予甲强龙冲击治疗。14例早期加用甲氨蝶呤,11例联合免疫球蛋白治疗。1例免疫抑制治疗3个月后因并发肺部严重感染出现呼吸衰竭,给予抗感染及呼吸机辅助呼吸后病情稳定;1例合并间质性肺损害者终因呼吸衰竭死亡;1例罕见的4岁女性钙质沉积者,经联合口服免疫抑制剂治疗后皮疹及皮肌炎症状缓解,但钙质沉积进行性加重;其余患儿临床均呈好转趋势。结论JDM是儿童期少见的炎性肌病,其以对称性的近端肌无力和特殊皮疹为特点,并发肺间质改变者死亡率较高。对于临床以皮疹、肌无力起病的患儿应考虑JDM。早期联合使用糖皮质激素、免疫抑制剂及静脉用人免疫球蛋白是治疗该病的关键,可明显改善预后。

Objective To investigate the clinical features, treatment, prognosis of juvenile dermatomyositis ( JDM ). Methods Clinical manifestations, laboratory data, treatment and prognosis of 20 inpatients with JDM were retrospectively analyzed. Results A total of 8 males and 12 females, with onset age of 1~10 years,( mean:5. 65 years) were included in this study. All patients had typical skin lesions and muscle weakness. Sixteen patients had Gottron' s papules, while 10 patients experienced systemic involvements, including respiratory system in 7 patients. All patients displayed higher levels of serum muscle enzymes. Elevated levels of creatine kinase and lactate dehydrogenase were observed in 13 and 18 cases, respectively. Electromyograms displayed myogenic abnormalities in all patients. Pathologic examination of muscle biopsies from 14 cases confirmed JDM. Glucocorticoids were given to 18 patients, including 2 patients received methylprednisolone ( MP ) pulse therapy. In addition to the treatments with glucocorticoids, 14 cases were also given orally methotrexate at beginning, and 11 cases received intravenous immunoglobulin ( IVIG ). One patient developed respiratory failure due to severe lung infections after 3 -month treatments with immunoglobulin. This patient was in stable condition following ant-infections and use of respiratory ventilator. One patients died of interstitial lung damage. In a rare case of 4 year old girl with calcification, treatments with immunosuppressant improved skin lesions and symptoms of myositis, but exacerbated calcification. All other cases showed some improvements. Conclusion JDM, a rare inflammatory myopathy in childhood, is manifested by proximal muscle weakness and pathognomonic rashes. The mortality rate is higher in patients complicated with interstitial lung damage. JDM should be considered in patients with muscle weakness and pathognomonic rashes. Treatments with the combination of glucocorticoids, immunosuppressants and IVIG can improve the prognosis.