Idiopathic pulmonary fibrosis (IPF) is a form of interstitial lung disease (ILD) characterized by abnormal wound healing in the lung that leads to progressive scarring and loss of lung function. The incidence of IPF is rising, with an estimated 2.8 to 18 cases per 100,000 people in North America alone. Pirfenidone and nintedanib are the only treatment options for IPF in the absence of underlying liver disease. These approved treatments slow the progression of IPF but cannot reverse the disease process. Being associated with severe adverse events it leads to treatment discontinuations. Several investigational, potentially disease-modifying therapeutic options are currently in development with the potential to halt or even reverse the disease process. In this digest, we have highlighted the recent advances of small pharma companies that are developing assets to reverse the disease process or overcome limitations of current approved therapies or improving QOL, etc.
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