Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive, chronic, irreversible form of fibrotic interstitial lung disease, with an estimated median survival of 3-5 years. Its prevalence has been estimated to be 0.7-63.0 per 100,000 with an incidence of 0.6-17.4 per 100,000 per year.
Recent research has begun to clarify the pathophysiology of IPF. While chronic inflammation was originally thought to be the underlying cause of this disease, it is now taken as evidence that IPF is the result of aberrant wound healing and a fibroproliferative cascade. Therefore, in the last decade, the development of novel therapeutic targets for IPF has shifted in this new direction. Till recently, the development of IPF therapies is mostly disappointing. However, the recent approval of two new therapies, pirfenidone and nintedanib, has markedly changed the landscape of IPF management, regardless, there is a long way to go in treating IPF. For example, most patients continue to progress despite treatment. Novel therapies that can modify the long-term course of IPF and, prognostic markers that predict survival, disease progression, and response to antifibrotic drugs are required. This review highlights the molecular and cellular mechanisms of IPF which explain the development of new therapies and provide an overview of existing therapies and novel therapeutics currently under investigation or in early clinical trials.Keywords: Aberrant repair process, antifibrotic therapy, disordered wound healing, idiopathic pulmonary fibrosis, myofibroblast, alveolar epithelial cell, transforming growth factor β1.
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