Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is among a group of interstitial lung diseases (ILDs) that are characterized by a combination of pulmonary inflammation and fibrosis. IPF is defined as having a radiographic or histological appearance of usual interstitial pneumonia (UIP), along with abnormal pulmonary function studies, and exclusion of other known causes of ILDs. IPF is associated with the worst prognosis of the ILDs with a mean life expectancy of approximately 3 years following diagnosis.

Overall rates of prevalence and incidence of IPF in the US are estimated to be 42.7 and 16.3 per 100,000 persons respectively. The Coalition for Pulmonary Fibrosis estimates that approximately 128,000 Americans currently have IPF, and approximately 48,000 new cases are diagnosed each year. Most people affected by IPF are over the age of 50, with approximately two-thirds of patients being diagnosed in their 60’s. The prevalence of IPF is higher in males than females. Most patients are symptomatic with dyspnea or cough for up to two years before the diagnosis is established. For more information please visit the websites of the Coalition for Pulmonary Fibrosis, the Pulmonary Fibrosis Foundation or the National Institutes of Health.

Lung Transplantation

Lung transplantation is the only effective treatment for several end-stage lung diseases, including, most commonly, idiopathic pulmonary fibrosis (IPF), chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), as well as other underlying lung diseases. Chronic rejection, known as bronchiolitis obliterans syndrome (BOS) is the leading cause of death in these patients, and outcomes after a lung transplantation procedure are poor, especially when compared with those after transplantation of other solid organs. Early mortality rates approach 15% in most series, and 5-year patient survival rates are approximately 50%.