医学
发病机制
肺
肺纤维化
纤维化
间质性肺病
疾病
病理生理学
特发性肺纤维化
病理
重症监护医学
内科学
作者
Thomas Koudstaal,Manuela Funke-Chambour,Michael Kreuter,Philip L. Molyneaux,Marlies Wijsenbeek
标识
DOI:10.1016/j.molmed.2023.08.010
摘要
Pulmonary fibrosis (PF) encompasses a spectrum of chronic lung diseases that progressively impact the interstitium, resulting in compromised gas exchange, breathlessness, diminished quality of life (QoL), and ultimately respiratory failure and mortality. Various diseases can cause PF, with their underlying causes primarily affecting the lung interstitium, leading to their referral as interstitial lung diseases (ILDs). The current understanding is that PF arises from abnormal wound healing processes triggered by various factors specific to each disease, leading to excessive inflammation and fibrosis. While significant progress has been made in understanding the molecular mechanisms of PF, its pathogenesis remains elusive. This review provides an in-depth exploration of the latest insights into PF pathophysiology, diagnosis, treatment, and future perspectives.
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