Definition
Idiopathic pulmonary fibrosis (IPF) is a chronic disease that causes inflammation (swelling and irritation) and fibrosis (scarring) of tissue in the lungs. It usually occurs in people between ages 50 and 70.
Causes
The word “idiopathic” in the name of this disease means the cause is not known. Researchers think that IPF involves an exaggerated and uncontrolled inflammatory response that produces the fibrous scar tissue. What starts the injury and abnormal healing is not known. Over time, the scarring surrounds the thin-walled small air sacs in the lungs (alveoli). This makes the tissues become thicker and stiffer. As a result, breathing becomes difficult, and the lungs gradually lose their ability to provide oxygen to the rest of the body.
What are the symptoms? Shortness of breath, particularly with exertion Chronic dry, hacking cough Fatigue and weakness Discomfort in the chest Loss of appetite Potential weight loss
What is the prevalence of Pulmonary Fibrosis?
There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually.
Shortness of breath, particularly with exertion