Interstitial lung disease (ILD) is common in idiopathic inflammatory myopathies in adults, especially in patients with antisynthetase syndrome and anti-MDA5 antibody-associated dermatomyositis. Pulmonary manifestations can range from subclinical ILD to rapidly progressive respiratory failure. Coexistent myositis, characteristic skin lesions, arthritis, and Raynaud's phenomenon are common. However, 16–65% of patients present with isolated lung disease. Detection of myositis-specific and myositis-associated antibodies can aid in diagnosis and disease characterisation. Chest imaging and pathology most commonly show non-specific interstitial pneumonia and organising pneumonia patterns. Immunosuppression is the mainstay of management with aggressive combination treatment for rapidly progressive disease and incremental escalation as needed for chronic ILD. The addition of antifibrotic agents is an option in progressive fibrotic disease, and lung transplantation can be considered in severe, end-stage disease. Most patients respond to treatment, but short-term mortality remains high for patients with rapidly progressive disease associated with anti-MDA5 antibody ILD.

中文翻译：

---

成人特发性炎症性肌病相关肺病


间质性肺病 （ILD） 常见于成人特发性炎性肌病，尤其是在抗合成酶综合征和抗 MDA5 抗体相关皮肌炎患者中。肺部表现范围从亚临床 ILD 到快速进展性呼吸衰竭。共存的肌炎、特征性皮损、关节炎和雷诺现象很常见。然而，16-65% 的患者表现为孤立性肺病。检测肌炎特异性抗体和肌炎相关抗体有助于诊断和疾病特征。胸部影像学检查和病理学检查最常显示非特异性间质性肺炎和机化性肺炎。免疫抑制是主要的治疗方法，对于快速进展的疾病，积极联合治疗，对于慢性 ILD 患者，应根据需要进行渐进式加重。对于进行性纤维化疾病，加用抗纤维化药物是一种选择，对于严重的终末期疾病，可以考虑肺移植。大多数患者对治疗有反应，但与抗 MDA5 抗体 ILD 相关的快速进展性疾病患者的短期死亡率仍然很高。