摘要
目的 探讨抗合成酶综合征的临床特征、诊断分类、治疗.方法 回顾分析1例抗合成酶综合征患儿的临床资料,并复习相关文献.结果 患儿女,8岁,以发热、肌无力为首发表现就诊,查体发现有机械手,实验室检查发现肌酶谱明显升高,检出肌炎特异性抗体抗Jo-1抗体,肌肉MRI双下肢软组织弥漫性水肿,血液净化、激素联合环磷酰胺治疗后明显好转.结论 抗合成酶综合征是以肌肉非化脓性炎症为主要特征的多系统自身免疫性疾病,易合并肺间质病变,肌炎特异性抗体有助于诊断及预后,早诊断、早治疗可改善预后并预防危重症的发生.
Objective To explore the clinical features,diagnosis,classification and treatment of antisynthetase syndrome.Methods The clinical data of one child with antisynthetase syndrome were retrospectively analyzed with relevant literature review.Results The child was 8 years old,with fever and muscle weakness as the starting performance.Physical examination found that she has manipulator,laboratory examination found that the muscle enzyme spectrum significantly increased,myositis specific antibody detected anti-Jo-1 antibody,muscle MRI of lower limbs showed soft tissue diffuse edema,blood purification,and hormone combined with cyclophosphamide treatment significantly improved the disease.Conclusion Antisynthetase syndrome is a multi-system autoimmune disease characterized by non-suppurative muscle inflammation,which is easily combined with pulmonary interstitial lesions.Myositis specific antibodies are helpful for diagnosis and prognosis.Early diagnosis and treatment can improve prognosis and prevent the occurrence of critical illness.
作者
张瑾
封其华
ZHANG Jin;FENG Qi-hua(Children's Hospital of Soochow University,Suzhou,Jiangsu,215003,China)
出处
《中国血液流变学杂志》
CAS
2020年第1期102-104,共3页
Chinese Journal of Hemorheology
关键词
抗合成酶综合征
肌无力
肌炎特异性抗体
儿童
antisynthetase syndrome
muscle weakness
myositis specific antibodies
child
