Idiopathic diffuse interstitial fibrosis
Overview
Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.
Symptoms
Chest pain (occasionally) Cough (usually dry) Decreased tolerance for activity Shortness of breath during activity that lasts for months or years and over time will also occur at rest
Causes
No one knows what causes pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening makes it increasingly difficult to breathe. In some people the disease gets worse quickly (over months to a few years), but other people have little worsening of the disease over time. The condition is believed to result from an inflammatory response to an unknown substance. "Idiopathic" means no cause can be found. The disease occurs most often in people between 50 - 70 years old.
Diagnosis
Some patients may improve when they are treated with corticosteroids or cytotoxic drugs, but in most people the disease can get worse even with treatment. This worsening can happen quickly, or very slowly.
Treatment
No known cure exists for idiopathic pulmonary fibrosis. Medications such as corticosteroids and cytotoxic drugs may be given to reduce swelling (inflammation), but these treatments usually don't work. Oxygen is given to patients who have low blood oxygen levels. Some patients with advanced pulmonary fibrosis may need a lung transplant. Lung rehabilitation will not cure the lung disease, but it can help maintain exercise capacity.