Diffuse parenchymal lung disease; Alveolitis; Cryptogenic fibrosing alveolitis (CFA); Idiopathic pulmonary pneumonitis (IPP)
Diffuse interstitial lung disease refers to a group of lung disorders in which the deep lung tissues become inflamed.
The lungs contain tiny air sacs (alveoli), which is where oxygen is absorbed. These air sacs open up or expand with each breath.
The tissue around these air sacs is called the interstitium. In people with interstitial lung disease, this tissue becomes stiff or scarred, and the air sacs are not able to expand as much. As a result, not as much oxygen can get into your lungs, and therefore to your body.
Interstitial lung diseases can be broken down into two large groups:
There are several types of idiopathic ILD. Idiopathic pulmonary fibrosis (IPF) is the most common type. Less common types include:
There are dozens of different causes of ILD.
Cigarette smoking may increase the risk of developing some forms of ILD and may cause the disease to be more severe.
Shortness of breath is a key symptom of interstitial lung disease. People may breathe faster or need to take deep breaths.
Most people with this condition also have a dry cough. A dry cough means you do not cough up any mucus or sputum.
Over time, weight loss, muscle and joint pain, and fatigue are also present.
People with advanced ILD may have:
The following tests may be done:
Treatment depends on the cause of the disease. Anti-inflammatory drugs, such as corticosteroids or drugs that suppress the immune system, are prescribed if an autoimmune disease is causing the problem.
If there is no specific treatment for the condition, therapy is aimed at making you more comfortable and supporting lung function.
Some patients with advanced ILD may need a lung transplant.
For additional information and resources, see lung disease support group.
Chances of recovery or the disease getting worse depend on the cause, and how severe the disease was when it was first diagnosed.
Call your health care provider if:
Avoid exposure to substances known to cause lung disease.
Quitting smoking can prevent ILD from getting worse.
People who are heavily exposed to known causes of occupational lung disease in the workplace are usually routinely screened for lung disease. These jobs can include coal miners, sand blasters, and ship workers.
Goldman L, Ausiello D. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders; 2008.
American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus. Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;165:277-304.
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