Abstract：
Idiopathic pulmonary fibrosis (IPF) is the archetypal interstitial lung disease. It is a chronic progressive condition that is challenging to manage as the clinical course of the disease is often difficult to predict. The prevalence of IPF is rising globally and in Japan, where it is estimated to affect 27 individuals per 100,000 of the population. Greater patient numbers and the poor prognosis associated with IPF diagnosis mean that there is a growing need for disease management approaches that can slow or even reverse disease progression and improve survival. Considerable progress has been made in recent years, with the approval of two antifibrotic therapies for IPF (pirfenidone and nintedanib), the availability of Japanese treatment guidelines, and the creation of global and Japanese disease registries. Despite this, significant unmet needs remain with respect to the diagnosis, treatment, and management of this complex disease. Each of these challenges will be discussed in this review, including making a timely and differential diagnosis of IPF, uptake and adherence to antifibrotic therapy, patient access to pulmonary rehabilitation, lung transplantation and palliative care, and optimal strategies for monitoring and staging disease progression, with a particular focus on the status in Japan. In addition, the review will reflect upon how ongoing research, clinical trials of novel therapies, and technologic advancements (including artificial intelligence, biomarkers, and genomic classification) may help address these challenges in the future.
摘要：
特发性肺纤维化（IPF）是典型的间质性肺病。这是一种慢性进行性疾病，难以控制，因为该疾病的临床过程通常很难预测。IPF的患病率在全球和日本都在上升，据估计，每10万人中有27人受到影响。与IPF诊断相关的更多患者数量和不良预后意味着对可以减缓或甚至逆转疾病进展并提高生存率的疾病管理方法的需求日益增长。近年来取得了相当大的进展,随着两种抗纤维化治疗IPF（吡非尼酮和尼达尼布）的批准，日本治疗指南的可用性，以及全球和日本疾病登记处的建立。尽管如此,在诊断方面仍然存在大量未满足的需求，治疗,以及这种复杂疾病的管理。这些挑战中的每一个都将在本次审查中讨论，包括对IPF进行及时的鉴别诊断，摄取和坚持抗纤维化治疗，患者获得肺康复，肺移植和姑息治疗，以及监测和分期疾病进展的最佳策略，特别关注日本的地位。此外,审查将反映正在进行的研究，新疗法的临床试验，和技术进步(包括人工智能，生物标志物，和基因组分类)可能有助于解决未来的这些挑战。
