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Abstract：
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by irreversible scarring of lung tissue, leading to death. Despite recent advancements in understanding its pathophysiology, IPF remains elusive, and therapeutic options are limited and non-curative. This review aims to synthesize the latest research developments, focusing on the molecular mechanisms driving the disease and on the related emerging treatments. Unfortunately, several phase 2 studies showing promising preliminary results did not meet the primary endpoints in the subsequent phase 3, underlying the complexity of the disease and the need for new integrated endpoints. IPF remains a challenging condition with a complex interplay of genetic, epigenetic, and pathophysiological factors. Ongoing research into the molecular keystones of IPF is critical for the development of targeted therapies that could potentially stop the progression of the disease. Future directions include personalized medicine approaches, artificial intelligence integration, growth in genetic insights, and novel drug targets.
摘要：
特发性肺纤维化(IPF)是一种以肺组织不可逆瘢痕形成为特征的间质性肺病,导致死亡。尽管最近在理解其病理生理学方面取得了进展，IPF仍然难以捉摸，和治疗选择是有限的和非治愈的。这篇综述旨在综合最新的研究进展，关注驱动疾病的分子机制和相关的新兴治疗方法。不幸的是,几项2期研究显示有前景的初步结果未达到随后3期的主要终点,这是疾病的复杂性和对新的综合终点的需要.IPF仍然是一个具有挑战性的条件，具有复杂的遗传相互作用，表观遗传,病理生理因素。对IPF分子基石的持续研究对于开发可能阻止疾病进展的靶向疗法至关重要。未来的方向包括个性化医疗方法，人工智能集成，遗传洞察力的增长，和新的药物靶标。
