Fibrotic interstitial lung diseases (ILDs) are often challenging to diagnose and classify, but an accurate diagnosis has significant implications for both treatment and prognosis. A subset of patients with fibrotic ILD experience progressive deterioration in lung function, physical performance, and quality of life. Several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. Morphological similarities, common underlying pathobiologic mechanisms, and the consistently progressive worsening of these patients support the concept of a progressive fibrosing (PF)-ILD phenotype that can be applied to a variety of ILD subtypes. The conventional approach has been to use antifibrotic medications in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive medications in patients with other fibrotic ILD subtypes; however, recent clinical trials have suggested a favourable treatment response to antifibrotic therapy in a wider variety of fibrotic ILDs. This review summarizes the literature on the evaluation and management of patients with PF-ILD, and discusses questions relevant to applying recent clinicial trial findings to real-world practice.

中文翻译：

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纤维化间质性肺疾病的进展。

纤维化间质性肺疾病（ILD）常常难以诊断和分类，但是准确的诊断对治疗和预后都有重要意义。一部分患有纤维化ILD的患者经历了肺功能，身体机能和生活质量的逐步恶化。已经报道了几种ILD进展的危险因素，例如男性，年龄较大，基线肺功能低下以及通常的间质性肺炎的放射学或病理学模式。这些患者的形态相似性，常见的潜在病理生物学机制以及持续进展的病情恶化支持可以应用于多种ILD亚型的进行性纤维化（PF）-ILD表型的概念。常规方法是对特发性肺纤维化（IPF）患者使用抗纤维化药物，对其他纤维化ILD亚型患者使用免疫抑制药物。但是，最近的临床试验表明，在多种纤维化ILD中，对抗纤维化治疗的治疗反应良好。这篇综述总结了有关PF-ILD患者评估和治疗的文献，并讨论了与将最新临床试验结果应用于现实世界有关的问题。