Overview

Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease characterized by a progressive and irreversible decline in lung function. It is a progressive and ultimately fatal disease that causes scarring and thickening of the lung tissue leading to respiratory failure.

The causes of IPF remains unknown; however, some risk factors include cigarette smoking, gastroesophageal reflux, specific environmental exposures, viral infection and age. A genetic predisposition may also be a factor. (Drugs R D. 2018 Mar; 18(1): 19–25.

IPF is believed to be the result of an aberrant wound healing process including/involving abnormal and excessive deposition of collagen (fibrosis) in the pulmonary interstitium with minimal associated inflammation.

Treatments

In recent years both Pirfenidone and Nintedanib have been approved for use in multiple countries for treatment of IPF. Pirfenidone (5-methyl-1-phenyl-2-[1H]- pyridone) is an orally administered drug with antifibrotic, anti-inflammatory, and antioxidant effects as shown in preclinical studies (Core Evid. 2016; 11: 11–22) Nintedanib is a tyrosine kinase inhibitor. Both drugs have been shown to slow idiopathic pulmonary fibrosis progression and have an acceptable tolerability profile.

Despite the moderate success of Pirfenidone and Nintedanib, additional treatment options are needed and lung fibrosis remains a major unmet medical need.

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