摘要
抗黑色素瘤分化相关基因5(MDA5)抗体阳性的皮肌炎(DM)是炎性肌病的特殊亚型,较易合并快速进展的肺间质病变(RPILD),死亡率高。遗传和环境因素可能共同参与了该病的致病过程;临床表现、呼吸生理参数、影像学特征及血清学标记物可用来判断RPILD发生风险及评估预后。对于MDA5+DM-RPILD目前尚缺乏基于高质量循证医学证据的治疗策略,主流治疗方法是激素+钙调神经磷酸酶抑制剂+环磷酰胺的“三联疗法”和基于JAK抑制剂的方案,早期诊断、准确分层以及个体化治疗是获得良好预后的关键。
Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis(anti-MDA5+DM)is a special subtype of idiopathic inflammatory myopathy,which is significantly associated with rapidly progressive interstitial lung disease(RPILD),resulting in high mortality rates.Both genetic and environmental factors are supposed to play a role in the development of the disease.Clinical manifestations,respiratory physiology parameters,imaging features and serological markers are indicators of disease risk and prognosis.Nowadays,triple therapy(combination of glucocorticoids,tacrolimus,and intravenous cyclophosphamide)and JAK inhibitor-based therapy are the mainstream treatment strategy for MDA5+DM-RPILD,but overall lack high quality evidence.Early diagnosis,accurate risk stratification and individualized treatment are essential for a favorable outcome.
作者
姜丽丽
刘娜
段利华
JIANG Li-li;LIU Na;DUAN Li-hua(Department of Rheumatology and Clinical Immunology,Jiangxi Provincial People's Hospital,the First Affiliated Hospital of Nanchang Medical College,Jiangxi Nanchang 330006,China;Medical College of Nanchang University,Jiangxi Nanchang 330006,China)
出处
《内科急危重症杂志》
2023年第2期106-111,共6页
Journal of Critical Care In Internal Medicine
基金
国家自然科学基金(No:81960296,81871286)
江西省风湿免疫疾病临床医学研究中心(No.20192BCD42005)
江西省医学领先学科项目(风湿病学与自体免疫病学)
江西省医学学科省市共建项目(风湿病学与自体免疫学)。
关键词
皮肌炎
黑色素瘤分化相关基因5
快速进展性肺间质病变
Dermatomyositis
Melanoma differentiation-associated gene 5
Rapidly progressive interstitial lung disease
