Interstitial Lung Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK; National Heart and Lung Institute, Imperial College London, London, UK.
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy.
Lancet Respir Med. 2020 Sep;8(9):925-934. doi: 10.1016/S2213-2600(20)30355-6.
Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, which is known as the progressive fibrotic phenotype. Although the concept of progressive fibrosing ILD has been applied largely to patients with idiopathic pulmonary fibrosis (IPF), there is now an increasing focus on irreversible progressive fibrosis in a proportion of patients with a range of underlying ILD diagnoses. Evidence has emerged to support a possible role for antifibrotic therapy in these patients. In this Position Paper, we discuss the importance of retaining diagnostic scrutiny within the multidisciplinary team and suggest a multidomain definition for progressive fibrosis. We consider the potential role of antifibrotic drugs as second-line therapy in the treatment algorithm for patients with progressive non-IPF ILD. We highlight risk factors that might predispose individuals to developing progressive fibrosis. Finally, we discuss key uncertainties and future directions for research and clinical practice.
在纤维性间质性肺疾病（ILDs）的范围内，有一部分患者尽管接受了治疗，但肺部纤维化仍在不可避免地进展，这种情况被称为进行性纤维化表型。尽管进行性纤维性ILD 的概念主要应用于特发性肺纤维化（IPF）患者，但现在越来越关注一部分ILD 诊断患者的不可逆转的进行性纤维化。有证据表明，抗纤维化治疗可能对这些患者有一定作用。在本立场文件中，我们讨论了在多学科团队中保留诊断审查的重要性，并提出了进行性纤维化的多领域定义。我们考虑了在进行性非 IPFILD 患者的治疗算法中，将抗纤维化药物作为二线治疗的潜在作用。我们强调了可能导致个体发生进行性纤维化的风险因素。最后，我们讨论了研究和临床实践的关键不确定性和未来方向。
