Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is a serious disease of the lungs that involves fibrous tissue building in the lungs that lowers the ability of the lungs to provide Oxygen to the blood. The fibrous tissue most often has a honeycomb appearance and usually starts at the bottom of the lungs and works its way up as the disease progresses.

According to The Lung Institute, shortness of breath is the main symptom, and other symptoms often include coughing, weakness, fatigue, achy joints and weight loss. According to the NIH, studies have shown that most patients don’t survive 5 years after diagnosis with 2/3 of patients expiring between 3-5 years. 20% will survive more than 5 years and some of those live for many years as the disease progression is much slower for them. It is reported that there are approximately 135,000 IPF patients in the USA according to ICDA reporting. There are 34,000 new cases a year in the US. Prevalence is estimated more for men with 20.2/100,000 than women 13.2/100,000. The mean age at presentation is 66.

There does not appear to be any one cause for IPF. Idiopathic means unknown cause. It is called idiopathic pulmonary fibrosis because the vast majority of cases don’t have a known cause for it. Many doctors in the US believe it is due to chemical exposure including Asbestos. Many other doctors feel the cause can be attributed to radiation treatment, allergens, Myocplasms, molds and fungus infections. Often there appears to be a genetic component. The condition is characterized by an overexpression of profibrotic cytokines and relative deficiency of interferon gamma.

Another valuable source of information can be found from the Pulmonary Fibrosis Foundation located at: