Evaluating new treatment options.

Idiopathic pulmonary fibrosis (IPF) is the most prevalent type of idiopathic interstitial pneumonia, accounting for at least half of all diagnosed cases. Because it lacks a cure, the goal of treatment for IPF is to stabilize or reduce the rate of disease progression. Nonpharmacologic treatment options for IPF consist of long-term oxygen treatment, lung transplantation, and pulmonary rehabilitation. In the past, pharmacologic therapies for IPF included anticoagulants and anti-inflammatory or immunosuppressive agents. However, in late 2014, 2 therapies were approved by the US FDA for use in IPF: nintedanib and pirfenidone. While treatment of IPF was previously significantly impeded by a lack of effective agents and a paucity of clinical trial data on which to base guideline recommendations, these new agents provide notable breakthroughs in management of IPF, and continued research may break further, new fertile ground for management.