Ofev (Nintedanib): First Tyrosine Kinase Inhibitor Approved for the Treatment of Patients with Idiopathic Pulmonary Fibrosis. (2015)

Idiopathic pulmonary fibrosis, a debilitating lung disease, affects approximately 128,100 patients in the United States, with 48,000 new cases diagnosed annually.1 Idiopathic pulmonary fibrosis is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause that occurs primarily in older adults (ie, aged 50–75 years).2 The disease is characterized by progressive worsening of dyspnea (shortness of breath) and lung function, as well as a poor prognosis.2 In fact, the median survival for patients with idiopathic pulmonary fibrosis in the United States is 2.5 to 3.5 years after diagnosis, with the disease claiming an estimated 40,000 lives annually.3

The course and severity of idiopathic pulmonary fibrosis varies from person to person. Symptoms include dyspnea, dry cough, fatigue, unexplained weight loss, and aching muscles and joints.4 An estimated 9 in 10 patients with idiopathic pulmonary fibrosis also have gastroesophageal reflux disease.5 The symptoms of idiopathic pulmonary fibrosis can have a profound impact on an individual's physical activity, independence, and quality of life.4,6

Complications of idiopathic pulmonary fibrosis include pulmonary hypertension, right-sided heart failure (cor pulmonale), respiratory failure, and lung cancer.4 Acute exacerbations are associated with increased morbidity and mortality rates.6

According to a retrospective 2001–2008 claims database analysis, the total direct medical costs for people with idiopathic pulmonary fibrosis were $26,378 per person annually; furthermore, these costs were $12,124 higher than the costs incurred by age- and sex-matched control patients.7 During this same period, the all-cause hospital admission rates and the all-cause outpatient visits for patients with idiopathic pulmonary fibrosis were twice that of the control group.7

Patients with idiopathic pulmonary fibrosis may receive oxygen therapy and/or pulmonary rehabilitation. For some patients with severe disease, a lung transplantation may be a last-resort option if other treatment options fail.4

In the past, conventional medications for patients with idiopathic pulmonary fibrosis included glucocorticosteroids (ie, prednisone) or immunosuppressants.8 A 2-drug combination regimen of prednisone and azathioprine or a 3-drug regimen of prednisone, azathioprine, and N-acetylcysteine were used to treat the disease.8 However, based on the findings from the PANTHER-IPF study that was funded by the National Heart, Lung, and Blood Institute and published in 2012, treatment with the triple combination of prednisone, azathioprine, and N-acetylcysteine was shown to increase the rate of mortality and hospitalization compared with placebo.8

Until October 2014, no medications were approved by the US Food and Drug Administration (FDA) for the treatment of patients with idiopathic pulmonary fibrosis.9 Approaches that reduce the decline in forced vital capacity may slow the progression of this devastating disease and improve outcomes for affected patients.10