Medical Therapies for Idiopathic Pulmonary Fibrosis

If the results of pulmonary function tests indicate that you have mild to moderate idiopathic pulmonary fibrosis, your doctor may recommend medication. Two medications, pirfenidone and nintedanib, have been shown to delay the progression of lung function decline.

These medications are well tolerated by most people. Side effects of pirfenidone may include nausea and sensitivity to light. Nintedanib may cause diarrhea and nausea.

Oxygen therapy can help to improve blood oxygen levels in people with idiopathic pulmonary fibrosis, alleviating symptoms such as shortness of breath. Supplemental oxygen is stored in a metal cylinder or container that is connected to small tubes that fit in your nostrils or to a face mask. This is placed over your nose and mouth, allowing oxygen to be delivered to your lungs.

Initially, oxygen therapy may be needed only when you exercise, because your body demands more oxygen at this time. It may also be recommended for when you sleep. If your symptoms become more severe, your doctor may prescribe continuous oxygen therapy.

If idiopathic pulmonary fibrosis becomes severe, your NYU Langone doctor may recommend a lung transplant and refer you to experts at the NYU Langone Transplant Institute.