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王国春.特发性炎性肌病相关间质性肺疾病诊治进展[J].内科急危重症杂志,2026,32(2):116-120
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| DOI：10.11768/nkjwzzzz20260203 |
| 中文关键词: 特发性炎性肌病 肺间质病变 诊断与治疗 |
| 英文关键词: |
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| 摘要点击次数: 121 |
| 全文下载次数: 149 |
| 中文摘要: |
| 摘要 特发性炎性肌病相关间质性肺疾病（IIM-ILD）是特发性炎性肌病（IIM）常见且严重的并发症，显著影响患者生存质量与预后。本文基于 “临床-免疫-病理” 三维体系，将其分为抗合成酶综合征相关ILD(ASyS-ILD)、抗MDA5抗体阳性DM相关ILD（MDA5-DM-ILD）等五大亚型，各亚型在临床特征、病理表现上存在显著异质性。IIM-ILD的发病机制尚不明确，与遗传、环境因素及免疫紊乱密切相关。IIM-ILD治疗需个体化，以糖皮质激素联合免疫抑制剂为基础，难治性病例可采用生物制剂等治疗。部分患者抗纤维化与感染预防亦为治疗的重要部分。未来需通过精准医学手段优化诊治方案，改善患者预后。 |
| 英文摘要: |
| Abstract Interstitial lung disease associated with idiopathic inflammatory myopathy (IIM-ILD) is a common and severe complication of IIM, which significantly impairs patients’ quality of life and prognosis. Based on the three-dimensional system of "clinical-immunological-pathological" features, this disease is classified into five major subtypes including ASyS-ILD and MDA5-DM-ILD etc., with distinct heterogeneity in clinical characteristics and pathological manifestations among these subtypes. The pathogenesis of IIM-ILD remains unclear, but it is closely associated with genetic factors, environmental triggers, and immune dysregulation. Individualized treatment is required for IIM-ILD, which is based on glucocorticoids combined with immunosuppressants. For refractory cases, biological agents and other therapies can be adopted. In addition, anti-fibrotic treatment and infection prevention are also important components of the management strategy. In the future, precision medicine approaches are needed to optimize diagnosis and treatment protocols, thereby improving patients' prognosis. |
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