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Progress in the treatment of pulmonary fibrosis.
The Lancet Respiratory Medicine ( IF 76.2 ) Pub Date : 2020-03-05 , DOI: 10.1016/s2213-2600(20)30062-x
Marlies Wijsenbeek 1
Affiliation  

Pulmonary fibrosis encompasses a wide range of fibrosing interstitial lung diseases (ILDs) with different underlying causes. In the past two decades, most focus has been on idiopathic pulmonary fibrosis (IPF), the most prevalent and relentlessly progressive type of fibrosing ILD. For other forms of fibrosing ILD, data on the proportion of patients who develop progressive pulmonary fibrosis are still scarce and depend on the disease studied and criteria used. Guidelines seem dedicated to assessing the likelihood of IPF versus another fibrosing ILD, as treatment, trial options, and prognosis differ. For the treatment of IPF, anti-fibrotic therapy in the form of nintedanib or pirfenidone is recommended, whereas, other forms of fibrosing ILD are mostly treated with immunosuppressive medication. This focus on IPF versus other fibrosing ILDs is likely to shift with the progress made in the ILD-field. Communalities in underlying pathobiological mechanisms as well as disease behaviour have been reported for different forms of pulmonary fibrosis regardless of the initial underlying cause that sets off the disease. Furthermore, three trials have shown response to anti-fibrotic therapy in patients with various forms of progressive fibrosing ILD, by use of different criteria to define progression. Combining two out of three domains of forced vital capacity (FVC), high resolution CT, and symptoms (INBUILD criteria) allowed for identification of patients with fibrosing ILD with a progressive phenotype similar to IPF. This pragmatic approach of identifying patients with progressive fibrosing ILD is feasible in almost every setting and close to daily practice in which we include more parameters than FVC to assess disease progression.

中文翻译:

肺纤维化的治疗进展。

肺纤维化涵盖了多种具有不同潜在病因的纤维化间质性肺疾病(ILD)。在过去的二十年中,最关注的焦点是特发性肺纤维化(IPF),这是纤维化ILD的最普遍和无情的发展类型。, 对于其他形式的纤维化ILD的,对谁开发逐步肺纤维化患者的比例数据依然匮乏,依赖于研究和使用标准的疾病。由于治疗,试验选择和预后不同,指南似乎专门用于评估IPF与另一纤维化ILD的可能性。, 对于IPF的治疗,建议采用nintedanib或pirfenidone形式的抗纤维化治疗,而其他形式的纤维化ILD大多采用免疫抑制药物治疗。随着IPL领域的进展, 对IPF和其他纤维化ILD的关注可能会发生转变。已经报道了不同形式的肺纤维化的潜在病理生物学机制以及疾病行为方面的共同点,而与引起该疾病的最初潜在原因无关。此外,三项试验显示,通过使用不同的标准定义进展情况,各种形式的进行性纤维化ILD患者对抗纤维化治疗的反应。,  ,  , 结合三个域的强制肺活量(FVC),高分辨率CT和症状(已建立标准)中的两个域,可以鉴定纤维化ILD的进展性表型类似于IPF的患者。这种实用的识别进行性纤维化ILD患者的方法几乎在每种情况下都是可行的,并且接近于日常实践,在该实践中,我们包括比FVC更多的参数来评估疾病进展。
更新日期:2020-03-06
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