姜丽丽.MDA5抗体阳性皮肌炎合并快速进展性肺间质病变的诊治进展[J].内科急危重症杂志,2023,29(2):106-111
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| DOI：10.11768/nkjwzzzz20230204 |
| 中文关键词: 皮肌炎 黑色素瘤分化相关基因5 快速进展性肺间质病变 |
| 英文关键词: |
| 基金项目: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） |
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| 中文摘要: |
| 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。 |
| 英文摘要: |
| 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. |
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