Matthew C. Lucas, David C. Budd
Abstract Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by scarring of the lung interstitium that likely occurs because of repetitive wounding of the alveolar epithelium, followed by incomplete healing of the injury. IPF is a disease of high, unmet medical need due to an exceptionally poor prognosis combined with a lack of effective treatment options capable of altering its progression. There is an urgent need for the development of novel pharmacological agents capable of providing significant efficacy while modifying the natural course of the disease. Herein, we describe emerging targets for the treatment of IPF and progress in the development of small-molecule modulators against them. Targets for which there is limited pharmacologic validation or against which only biologic modalities exist are excluded.
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