幼年皮肌炎94例临床分析及影响幼年皮肌炎预后的危险因素分析
发布时间：2018-09-14 15:10
【摘要】：背景和目的幼年皮肌炎是一种少见的风湿免疫性疾病,也是儿童时期最常见的炎性肌病,其以对称性的近端肌无力和特殊皮疹为特点,其他系统亦可受累。通过分析94例幼年皮肌炎患儿的病例特点及随访情况,总结幼年皮肌炎患儿的临床特点,提出影响幼年皮肌炎预后的可能因素。方法选取1990年至2016年在北京协和医院住院诊治的幼年皮肌炎患儿,回顾性分析患儿的人口学特征、临床表现、辅助检查结果、治疗情况、随诊情况及病程中出现的并发症。根据随诊情况将患儿分为单病程组与预后不良组,通过统计学方法分析,得出影响幼年皮肌炎患儿预后的可能危险因素。结果本研究共纳入符合诊断标准的病例94例,其中经典型皮肌炎90例,临床无肌病皮肌炎4例,其中2例病例因合并肺间质病变、肺部感染死亡。坚持随访3年以上病例44例,最长随访时间194个月,31人达到临床缓解,临床缓解率为70.45%,达到临床缓解的平均时间为7.00(4.50-17.00)个月,其中15人(34.09%)临床治愈,达到临床治愈的平均时间为50.46个月;8人(18.18%)达到临床缓解,但口服药物至少有一种尚未减停;8人(18.18%)在药物减量过程中曾出现疾病复发,但末次复发后可满足临床缓解标准;12人(27.27%)病程迁延,始终无法达到临床缓解;1例(2.27%)激素减量过程中合并EBV感染,继发噬血细胞综合征。将病情迁延、病情反复、死亡病例纳入预后不良组,与单病程组进行比较,预后不良组平均起病年龄6.05±3.21岁,单一病程组平均起病年龄6.77±3.40岁,但无统计学差异;预后不良组确诊至起病平均时间5.5个月,单病程组4个月,无统计学差异;存在呼吸系统受累共10例,其中预后不良组8例,单病程组2例,有统计学差异(p0.05);病程中出现皮下钙化共16例,其中预后不良组11例,单病程组5例,有统计学差异(p0.05)。将起病年龄、未治疗时间、是否出现皮下钙化、是否存在呼吸系统受累共同进行Logistic回归分析,其中存在呼吸系统受累两组间存在显著差异(p0.05)。结论幼年皮肌炎可呈现多系统损害,多数病例预后良好,70%以上病例可达到临床缓解,呼吸系统受累是幼年皮肌炎预后不良的危险因素,幼年皮肌炎患者无论有无呼吸系统临床症状均应完善呼吸系统评估。
[Abstract]:Background and objective infantile dermatomyositis is a rare rheumatic immune disease and the most common inflammatory myopathy in childhood. It is characterized by symmetrical proximal myasthenia and special rash. Other systems may also be involved. The clinical features of 94 cases of infantile dermatomyositis and their follow-up were analyzed, and the possible factors influencing the prognosis of juvenile dermatomyositis were put forward. Methods Children with infantile dermatomyositis, who were hospitalized in Peking Union Hospital from 1990 to 2016, were retrospectively analyzed for their demographic characteristics, clinical manifestations, auxiliary examination results, treatment, follow-up and complications in the course of disease. The children were divided into single course group and poor prognosis group according to the follow up. The possible risk factors affecting the prognosis of infant dermatomyositis were obtained by statistical analysis. Results 94 cases were included in this study, including 90 cases of classic dermatomyositis and 4 cases of clinical non-myopathy dermatomyositis. 2 cases died of pulmonary infection due to pulmonary interstitial disease. 44 cases were followed up for more than 3 years. The longest follow-up time was 194 months. The clinical remission rate was 70.45, and the average time to clinical remission was 7.00 (4.50-17.00) months. Among them, 15 patients (34.09%) were cured clinically. The average time to achieve clinical cure was 50.46 months or 8 (18.18%), but at least one oral drug had not been reduced or stopped. 8 (18.18%) had relapsed in the course of drug reduction. However, 12 patients (27.27%) could meet the criteria of clinical remission after the last relapse, and the course of disease was delayed, and one case (2.27%) with EBV infection and secondary hemophagocytic syndrome was unable to achieve clinical remission. The average onset age of poor prognosis group was 6.05 卤3.21 years old, and the average onset age of single course group was 6.77 卤3.40 years, but there was no statistical difference. The mean time from diagnosis to onset was 5.5 months in poor prognosis group and 4 months in single course group, and there were 10 cases of respiratory system involvement, including 8 cases in poor prognosis group and 2 cases in single course group. There were 16 cases of subcutaneous calcification in the course of the disease, including 11 cases of poor prognosis group and 5 cases of single course group (p0.05). The onset age, untreated time, subcutaneous calcification and respiratory system involvement were analyzed by Logistic regression analysis. There was significant difference between the two groups (p0.05). Conclusion Young dermatomyositis may present multiple system damage, and the prognosis of most of the cases can reach clinical remission in more than 70% cases. Respiratory system involvement is the risk factor of poor prognosis of juvenile dermatomyositis. Patients with juvenile dermatomyositis should improve the assessment of respiratory system regardless of the clinical symptoms of respiratory system.
【学位授予单位】：北京协和医学院
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R725.9
本文编号：2243070
[Abstract]:Background and objective infantile dermatomyositis is a rare rheumatic immune disease and the most common inflammatory myopathy in childhood. It is characterized by symmetrical proximal myasthenia and special rash. Other systems may also be involved. The clinical features of 94 cases of infantile dermatomyositis and their follow-up were analyzed, and the possible factors influencing the prognosis of juvenile dermatomyositis were put forward. Methods Children with infantile dermatomyositis, who were hospitalized in Peking Union Hospital from 1990 to 2016, were retrospectively analyzed for their demographic characteristics, clinical manifestations, auxiliary examination results, treatment, follow-up and complications in the course of disease. The children were divided into single course group and poor prognosis group according to the follow up. The possible risk factors affecting the prognosis of infant dermatomyositis were obtained by statistical analysis. Results 94 cases were included in this study, including 90 cases of classic dermatomyositis and 4 cases of clinical non-myopathy dermatomyositis. 2 cases died of pulmonary infection due to pulmonary interstitial disease. 44 cases were followed up for more than 3 years. The longest follow-up time was 194 months. The clinical remission rate was 70.45, and the average time to clinical remission was 7.00 (4.50-17.00) months. Among them, 15 patients (34.09%) were cured clinically. The average time to achieve clinical cure was 50.46 months or 8 (18.18%), but at least one oral drug had not been reduced or stopped. 8 (18.18%) had relapsed in the course of drug reduction. However, 12 patients (27.27%) could meet the criteria of clinical remission after the last relapse, and the course of disease was delayed, and one case (2.27%) with EBV infection and secondary hemophagocytic syndrome was unable to achieve clinical remission. The average onset age of poor prognosis group was 6.05 卤3.21 years old, and the average onset age of single course group was 6.77 卤3.40 years, but there was no statistical difference. The mean time from diagnosis to onset was 5.5 months in poor prognosis group and 4 months in single course group, and there were 10 cases of respiratory system involvement, including 8 cases in poor prognosis group and 2 cases in single course group. There were 16 cases of subcutaneous calcification in the course of the disease, including 11 cases of poor prognosis group and 5 cases of single course group (p0.05). The onset age, untreated time, subcutaneous calcification and respiratory system involvement were analyzed by Logistic regression analysis. There was significant difference between the two groups (p0.05). Conclusion Young dermatomyositis may present multiple system damage, and the prognosis of most of the cases can reach clinical remission in more than 70% cases. Respiratory system involvement is the risk factor of poor prognosis of juvenile dermatomyositis. Patients with juvenile dermatomyositis should improve the assessment of respiratory system regardless of the clinical symptoms of respiratory system.
【学位授予单位】：北京协和医学院
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R725.9
【参考文献】
相关期刊论文 前1条
1 舒晓明;王国春;;炎性肌病的临床评估工具介绍[J];中华风湿病学杂志;2011年07期
，本文编号：2243070
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