论文部分内容阅读
目的研究幼年皮肌炎(JDM)的临床特征、实验室及辅助检查、治疗用药和疗效以及远期预后,使该病能早期诊断及时治疗。方法回顾性分析了2003—2007年北京儿童医院住院JDM患儿46例,分析其临床表现、实验室及影像学检查、治疗用药及远期随访结果和预后。结果46例患儿中女20例,男26例,女比男为1∶1.3;年龄1~14岁,平均年龄7岁。临床表现主要为皮疹及肌无力,93%的患儿有高春征,100%的患儿有颜面部紫红色皮疹,89%的患儿有甲床毛细血管异常;所有的患儿均有不同程度的肌无力,严重者需要呼吸机辅助呼吸。46%的患儿有内脏受累,其中大部分(71%)为2个以上系统受累,最常受累系统为呼吸系统(37%),其次是消化系统(33%)、心血管系统(26%)及神经系统(11%)。实验室检查100%的患儿有肌酶增高,其中CK最具特异性。所有的患儿肌电图均表现为肌源性损害。治疗用药,除应用糖皮质激素外,均在早期加用甲氨蝶呤,有肺损害及重症患儿加用环孢素A。早期治疗效果及远期预后均较好,46例患儿在急性期死亡2例,死因为肺部受累合并感染致呼吸衰竭,远期并发症少见。结论JDM是一类预后相对较好的自身免疫性疾病,关键在于早期诊断和积极治疗,认识JDM的特征性表现有助于早期诊断。
Objective To study the clinical features of young dermatomyositis (JDM), laboratory and laboratory examinations, treatment and efficacy and long-term prognosis, so that the disease can be diagnosed and treated promptly. Methods A retrospective analysis of 46 children hospitalized with JDM in Beijing Children’s Hospital from 2003 to 2007 was conducted to analyze their clinical manifestations, laboratory and imaging findings, therapeutic drug use and long-term follow-up results and prognosis. Results Among the 46 children, 20 were women, 26 were males and 1 to 1.3 females compared with males. The patients were from 1 to 14 years old with a mean age of 7 years. Clinical manifestations mainly rash and muscle weakness, 93% of children with high spring levy, 100% of children had a facial purple rash, 89% of children with nail bed capillary abnormalities; all children have varying degrees of Muscle weakness, severe ventilator-assisted breathing. Forty-six percent of children had visceral involvement, the majority of whom (71%) had more than two systems involved, the most commonly affected being respiratory (37%) followed by digestive (33%), cardiovascular (26% ) And nervous system (11%). Laboratory tests 100% of children with increased muscle enzymes, of which CK is the most specific. All children’s EMG showed myogenic damage. Treatment medication, in addition to glucocorticoid, are added in the early methotrexate, with lung damage and severe children with cyclosporine A. Early treatment and long-term prognosis are good, 46 cases of children died in the acute phase in 2 cases, the cause of death due to lung infection complicated with respiratory failure, long-term complications rare. Conclusions JDM is a kind of autoimmune disease with relatively good prognosis. The key lies in early diagnosis and active treatment. Recognizing the characteristic manifestation of JDM is helpful for early diagnosis.