Interstitial pulmonary lung disease (ILD), also known as interstitial pulmonary fibrosis, is a general term that describes more than 100 chronic lung disorders that damage the interstitium. The tissue located between the airs sacs of the lungs is called the interstitium.
The disease affects the lungs in three ways. First, the lung tissue is damaged. Second, the interstitium becomes inflamed. Finally, fibrosis (scarring) begins in the alveoli (air sacs) and interstitium, and the lung becomes stiff, making it difficult to breathe.
Lung scarring is irreversible. Corticosteroid drugs, the most common treatment, can sometimes slow the damage of interstitial lung disease. However, many patients never regain full use of their lungs.
While most cases of interstitial lung disease develop gradually with few warning signs, it may develop suddenly in some patients. Although doctors can pinpoint why some cases of interstitial lung disease occur, most are idiopathic (have no known cause). The most common type of idiopathic ILD is called interstitial pneumonitis, which causes patches of inflammation in the lung without infection.
More than 200,000 people have been diagnosed with ILD in the United States, and nearly five million people have been diagnosed worldwide. It is estimated that more than 40,000 patients die each year from ILD worldwide. The outlook for patients with ILD varies depending on the underlying cause of the disease.
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