Current and emerging drugs for idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis is a chronic, progressive and often fatal form of interstitial lung disease. It is characterized by injury with loss of lung epithelial cells and abnormal tissue repair, resulting in abnormal accumulation of fibroblasts and myofibroblasts, deposition of extracellular matrix and distortion of lung architecture, leading to respiratory failure. This lethal lung disorder continues to pose major clinical challenges as an effective therapeutic regimen has yet to be developed. In this report, therapeutic strategies are reviewed, including the use of antifibrotic agents, inhibition of cytokines, leukotrienes and cytokines receptors, and molecular targeting of specific signaling pathways during fibrotic processes and angiogenesis. This article examines the body of evidence supporting present therapies and reviews the newer agents being tested in patients with idiopathic pulmonary fibrosis.

Keywords::

• angiogenesis
• antifibrotic agents
• anti-inflammatory agents
• fibrocytes
• idiopathic pulmonary fibrosis
• IFN-γ
• inflammation
• myofibroblasts
• N-acetylcysteine
• pathogenesis
• pirfenidone
• repair

Acknowledgements

The authors would like to acknowledge CM Freeman, PhD, for critical review of manuscript and excellent artwork of RG Kunkel for her artistic contributions to this review.

Notes