[1]唐鲜艳,冯媛,彭悦,等.幼年皮肌炎20例临床特点及转归分析[J].中国皮肤性病学杂志,2019,(10):1153-1157.[doi:10.13735/j.cjdv.1001-7089.201809121]
 TANG Xianyan,FENG Yuan,PENG Yue,et al.Analysis of Clinical Characteristics and Prognosis in 20 Juvenile Dermatomyositis[J].The Chinese Journal of Dermatovenereology,2019,(10):1153-1157.[doi:10.13735/j.cjdv.1001-7089.201809121]
点击复制

幼年皮肌炎20例临床特点及转归分析
分享到:

《中国皮肤性病学杂志》[ISSN:1001-7089/CN:61-1197/R]

卷:
期数:
2019年10期
页码:
1153-1157
栏目:
临床经验
出版日期:
2019-09-13

文章信息/Info

Title:
Analysis of Clinical Characteristics and Prognosis in 20 Juvenile Dermatomyositis
文章编号:
1001-7089(2019)10-1153-05
作者:
唐鲜艳冯媛彭悦李小青
西安交通大学医学院附属儿童医院风湿免疫科,陕西 西安 710002
Author(s):
TANG XianyanFENG YuanPENG YueLI Xiaoqing
(partment of Immunology,Children's Hospital Affiliated Medical College of Xi'an Jiaotong University,Xi'an 710002,China)
关键词:
儿童 皮肌炎 临床特点
Keywords:
Children Juvenile dermatomyositis Clinical features
分类号:
R 593.26
DOI:
10.13735/j.cjdv.1001-7089.201809121
文献标志码:
B
摘要:
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。
Abstract:
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.

参考文献/References:

[1] Lundberg IE,Miller FW,Tjarnlund A,et al.Diagnosis and classification of idiopathic inflammatory myopathies[J].J Intern Med,2016,280(1):39-51.
[2] Ernste FC,Reed AM.Recent advances in juvenile idiopathic inflammatory myopathies [J].Curr Opin Rheumatol,2014,26(6):671-678.
[3] Martin N,Krol P,Smith S,et al.A national registry for juvenile dermatomyositis and other paediatric idiopathic inflammatory myopathies:10 years' experience,the Juvenile Dermatomyositis National(UK and Ireland )Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies[J].Rheumatology(Oxford),2011,50(1):137-145.
[4] Marie I,Hatron PY,Dominique S,et al.Short-term and long-term outcomes ofinterstitial lung disease in polymyositis and dermatomyositis:a series of 107 patient[J].Arthritis and Rheum,2011,63(11):3439-3447.
[5] Sun C,Lee JH,Yang YH,et al.Juvenile dermatomyositis:a 20-year retrospective analysis of treatment and clinical outcomes [J].Pediatrics and neonatology,2015,56(1):31-39.
[6] Sanner H,Sjaastad I,Flato B.Disease activity and prognostic factors in juvenile dermatomyositis:a long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool [J].Rheumatology(Oxford),2014,53(9):1578-1585.
[7] Gowdie PJ,Allen RC,Kornberg AJ,et al.Clinical features and disease course of patients with juvenile dermatomyositis[J].Int J Rheum Dis,2013,16(5):561-567.
[8] 谈丹丹,杨海坡,张艳芝,等.病理表现为炎症性肌病的LMNA相关先天性肌营养不良4例的表型/基因型研究[J].中华实用儿科临床杂志,2013,28(21):1660-1664.
[9] Mastaglia FL,Zilko PJ.Inflammatory myopathies:How to treat the difficult case[J].Clin Neurosci,2003,10(1):99-101.
[10] Enders FB,Bader-Meunier B,Baildam E,et al.Consensus-based recommendations for the management of juvenile dermatomyositis[J].Ann Rheum Dis,2017,76(2):329-340.
[11] Spencer CH,Rouster-Stevens K,Gewanter H,et al.Biologic therapies for refractory juvenile dermatomyositis:five years of experience of the Childhood Arthritis and Rheumatology Research Alliance in North America[J].Pediatr Rheumatol Online J,2017,15(1):1-8.

相似文献/References:

[1]黄云丽,张道军,李红宾,等.昆明地区儿童浅部真菌病1646例分析[J].中国皮肤性病学杂志,2012,(08):698.
 HUANG Yun-li,ZHANG Dao-jun,LI Hong-bin,et al.Analysis of 1 646 Cases of Children Superficial Mycosis in Kunming Region[J].The Chinese Journal of Dermatovenereology,2012,(10):698.
[2]冯进云,罗珍,王昊,等.儿童猩红热87例临床表现及皮疹特征[J].中国皮肤性病学杂志,2012,(08):702.
 FENG Jin-yun,LUO Zhen,WANG Hao,et al.Clinical Manifestations and Characteristic Skin Rashes in Children with Scarlet Fever:Analysis of 87 Cases[J].The Chinese Journal of Dermatovenereology,2012,(10):702.
[3]梁源,张立新,李楠,等.儿童中毒性表皮坏死松解症15例临床分析[J].中国皮肤性病学杂志,2015,(05):472.[doi:10.13735/j.cjdv.1001-7089.201405147]
 LIANG Yuan,ZHANG Li xin,LI Nan,et al.Clinical Analysis of 15 Pediatric Cases with Toxic Epidermal Necrolysis[J].The Chinese Journal of Dermatovenereology,2015,(10):472.[doi:10.13735/j.cjdv.1001-7089.201405147]
[4]胡伟,马慧群,闫小宁,等.左西替利嗪联合地氯雷他定治疗儿童慢性荨麻疹多中心临床观察[J].中国皮肤性病学杂志,2015,(05):481.[doi:10.13735/j.cjdv.1001-7089.201501034]
 HU Wei,MA Hui qun,YAN Xiao ning,et al.The Multicenter Study of Sesloratadine Combined Levocetirizine Therapy on Chronic Urticaria in Children[J].The Chinese Journal of Dermatovenereology,2015,(10):481.[doi:10.13735/j.cjdv.1001-7089.201501034]
[5]荚琳琳,季必华.儿童常见性病的临床特点[J].中国皮肤性病学杂志,2015,(07):743.[doi:10.13735/j.cjdv.1001-7089.201409165]
 JIA Lin lin,JI Bi hua.Clinical Characteristics of Common Sexually Transmitted Diseases in Children[J].The Chinese Journal of Dermatovenereology,2015,(10):743.[doi:10.13735/j.cjdv.1001-7089.201409165]
[6]滕蔚,金艳,史同新.儿童获得性梅毒20例临床分析[J].中国皮肤性病学杂志,2015,(08):825.[doi:10.13735/j.cjdv.1001-7089.201401081]
 TENG Wei,JIN Yan,SHI Tong xin.Clinical Analysis of 20 Cases of Acquired Syphilis in Children[J].The Chinese Journal of Dermatovenereology,2015,(10):825.[doi:10.13735/j.cjdv.1001-7089.201401081]
[7]王敏华,艾志琼,黄玲,等.大理市3~19岁儿童及青少年特应性皮炎现况调查[J].中国皮肤性病学杂志,2015,(11):1184.[doi:10.13735/j.cjdv.1001-7089.201412051]
 WANG Min hua,AI Zhi qiong,HUANG Ling,et al.The Prevalence Study of Atopic Dermatitis among Children and Teenagers Aged 3~19 Years in Dali[J].The Chinese Journal of Dermatovenereology,2015,(10):1184.[doi:10.13735/j.cjdv.1001-7089.201412051]
[8]李明娥,申小平,陆洪光.儿童泛发性脓疱型银屑病并血小板增高1例[J].中国皮肤性病学杂志,2016,(02):180.[doi:10.13735/j.cjdv.1001-7089.201506152]
 LI Ming e,SHEN Xiao ping,LU Hong guang.A Case of Generalized Pustular Psoriasis with Thrombocytosis in a Child[J].The Chinese Journal of Dermatovenereology,2016,(10):180.[doi:10.13735/j.cjdv.1001-7089.201506152]
[9]任强强.复方甘草酸苷、米诺地尔酊联合液氮冷冻治疗儿童斑秃33例临床观察[J].中国皮肤性病学杂志,2016,(02):219.[doi:10.13735/j.cjdv.1001-7089.201411089]
 REN Qiang qiang.Clinical Observation on Compound Glycyrrhizin Combined with Minoxidil and Cryotherapy with Liquid Nitrogen in the Treatment of 33 Children with Alopecia Areata[J].The Chinese Journal of Dermatovenereology,2016,(10):219.[doi:10.13735/j.cjdv.1001-7089.201411089]
[10]慕珍珍,杨帆,张悦,等.维A酸类药物治疗儿童皮肤病的临床应用进展[J].中国皮肤性病学杂志,2016,(03):308.[doi:10.13735/j.cjdv.1001-7089.201411057]
 MU Zhen zhen,YANG Fan,ZHANG Yue,et al.Progress on Clinical Experiences of Tretinoids in the Treatment of Children with Skin Diseases[J].The Chinese Journal of Dermatovenereology,2016,(10):308.[doi:10.13735/j.cjdv.1001-7089.201411057]

备注/Memo

备注/Memo:
[作者单位] 西安交通大学医学院附属儿童医院风湿免疫科,陕西 西安 710002
[通讯作者] 李小青,E-mail:xa_lxq@163.com
更新日期/Last Update: 2019-09-12