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Pirfenidone and Nintedanib for Idiopathic Pulmonary Fibrosis: A New Drug Therapy

Priyanka Wani, Mohamed Teleb, Miraie Wardi, Hasan J. Salameh

Department of Internal Medicine, Texas Tech University Health Sciences Center, Paul L. Foster School of Medicine, El Paso, TX, USA

Med Sci Rev 2015; 2:41-48

DOI: 10.12659/MSRev.894308

Available online:

Published: 2015-05-21

ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a chronic debilitating disease with progressive decline in lung function. The incidence of IPF is higher in males, with median survival of 3–5 years after diagnosis. Recent clinical trials have shown success in effective treatment with pirfenidone and nintedanib. The purpose of this article was to review the literature and determine the safety and efficacy of pirfenidone and nintedanib in patients with IPF.
Pirfenidone is an oral drug with anti-fibrotic and anti-inflammatory properties. Three published phase III clinical studies have assessed pirfenidone in IPF (CAPACITY 004 and 006), assessed pirfenidone to confirm efficacy and safety in IPF (ASCEND), and a Japanese trials evaluated pirfenidone for mild-to-moderated IPF. The Japanese and ASCEND trials demonstrated that higher doses of pirfenidone decreased the rate of decline of FVC. The CAPACITY 004 and 006 showed that pirfenidone significantly reduced decline in forced vital capacity (FVC) compared with placebo. The efficacy data provided evidence of treatment benefit in patients with IPF.
Nintedanib is a tyrosine kinases inhibitor that inhibits the intracellular receptors of angiogenesis, including platelet-derived growth factor (PDGF), vascular endothelial growth factor (VEGF), and fibroblast growth factor (FGF). There have been 2 published trials to improve pulmonary fibrosis with BIBF 1120 (TOMORROW), and high dose in Idiopathic Pulmonary Fibrosis patients (INPULSIS) 1 and 2 evaluating nintedanib for IPF. Both studies assessed nintedanib 150 mg twice daily in IPF patients compared to a placebo group, demonstrating that nintedanib is efficacious in reducing the rate of decline of FVC in patients with IPF.

Keywords: Anti-Inflammatory Agents, idiopathic pulmonary fibrosis, Vital Capacity