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皮肌炎/多发性肌炎伴发间质性肺病的研究进展
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Abstract:
特发性炎症性肌病(IIM)中，肺间质改变是其中的一个重要特征，尤其多见于皮肌炎(DM)和临床无肌病性皮肌炎(CADM)患者。这些肺间质改变可分为快速进展型间质性肺病(RP-ILD)和慢性间质性肺炎(CIP)，其中RP-ILD患者会在短期内经历急性、进展性的弥漫性肺损伤，预后较差。由于疾病早期往往缺乏明显或有特异性的呼吸道症状，且肺功能检查在间质性肺病(ILD)诊断中的特异性和灵敏性有限，因此早期识别风险因素和实施筛查对于疾病管理至关重要。探寻DM相关间质性肺病(DM-ILD)的风险因素和临床特征，能够为疾病的诊断和治疗提供更多依据，有助于稳定甚至改善肺功能，进而改善患者的预后。
Interstitial lung involvement is a significant characteristic in idiopathic inflammatory myopathies (IIM), particularly prevalent among patients with dermatomyositis (DM) and clinically amyopathic dermatomyositis (CADM). These interstitial lung alterations can be categorized into rapidly progressive interstitial lung disease (RP-ILD) and chronic interstitial pneumonia (CIP). Patients with RP-ILD experience acute, progressive diffuse lung injury over a short period, leading to poor prognosis. Due to the early stages of the disease often lacking distinct or specific respiratory symptoms and the limited specificity and sensitivity of pulmonary function tests in the diagnosis of interstitial lung disease (ILD), early identification of risk factors and implementation of screening are crucial for disease management. Investigating the risk factors and clinical features associated with DM-related interstitial lung disease (DM-ILD) can provide additional evidence for the diagnosis and treatment of the disease, which may help stabilize or even improve lung function, thereby improving patient prognosis.
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