Idiopathic pulmonary fibrosis is a chronic condition in which lung tissue becomes irritated and scarred over time. Current medical research cannot explain what triggers the scarring, though a combination of genetic and environmental factors are probably involved. The condition causes progressively worsening symptoms of chest pain and coughing that can eventually lead to lung collapse. Doctors can try to manage the disorder with anti-inflammatory medications and oxygen therapy, but respiratory complications usually prove fatal within about five years of the onset of symptoms.
The lungs are comprised of tiny sacs, called alveoli, that fill with air to replenish the blood. In the case of idiopathic pulmonary fibrosis, the normally flexible alveoli become inflamed, restricting their ability to expand and contract. Chronic inflammation gradually leads to scarring that permanently prevents alveoli from inflating. Inflammation can spread quickly, affecting a large number of alveoli in one or both lungs over the course of a few months or years.
The exact cause of inflammation and scarring is unknown. Most doctors believe that exposure to environmental toxins, such as cigarette smoke, industrial chemicals, and silica dust, play a significant role in fibrosis. Radiation treatments and chemotherapy drugs for cancer are suspected to be involved in some cases. In addition, research suggests that some people may be genetically predisposed to developing the condition. The average age of patients who are diagnosed with idiopathic pulmonary fibrosis is about 60, and the risk of developing lung problems increases significantly with advancing age.
A person who has idiopathic pulmonary fibrosis may not be able to take deep breaths due to alveoli constriction. Shortness of breath tends to be the worst following physical exertion. A dry cough, fatigue, weakness, and chest pain develop over time. If the condition is not diagnosed and treated in its early stages, breathing problems can become severe enough to cause lung collapse, total respiratory failure, and heart complications.
The earlier idiopathic pulmonary fibrosis is detected, the easier it is to manage. A doctor can conduct a thorough physical examination, blood tests, and diagnostic imaging scans to look for a possible cause of breathing problems. Chest x-rays and computerized tomography scans can reveal the location and severity of tissue scarring, and blood tests may show signs of low blood-oxygen. Since an underlying cause usually cannot be confirmed, treatment decisions are made based on the seriousness of physical symptoms.
Corticosteroids and other anti-inflammatory drugs may help relieve irritation in some cases, but medications are not always effective. Patients have the best chances of overcoming symptoms by quitting smoking, maintaining a healthy diet, and engaging in guided, light exercise. If fibrosis worsens to the point that respiratory failure is certain, a patient may be scheduled for a lung transplant.
