Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease in which progressive scarring of the lung causes fibrosis and loss of lung function. People with IPF experience dry, persistent cough and progressive fatigue. Patients have a poor prognosis with median survival after diagnosis of only 3 years without effective treatment. Thus, the prognosis for IPF is worse than many types of cancer.
How many have IPF?
Approximately 50,000 new cases of IPF are diagnosed each year. Risk factors include age, gender, tobacco smoking, and family history of pulmonary fibrosis since some genes have been associated with IPF.
How is IPF treated?
Currently, two antifibrotic agents are approved by the FDA and EMA for the treatment of IPF, namely pirfenidone and nintedanib. However, neither of these are able to halt the disease, but merely slow down disease progression. Additionally, both compounds are associated with significant side effects and not all patients show good effect of the treatment.
How is IPF diagnosed?
IPF is diagnosed by lung function tests, a chest X-ray and a high-resolution CT scan to identify the fibrotic pattern associated with IPF. A lung biopsy may also be needed in order to make a diagnosis.