What’s desquamative interstitial pneumonia?

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Desquamative interstitial pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia that causes tissue destruction due to interstitial inflammation triggered by an increase in macrophage cells. Smoking is the most common cause, and symptoms include chronic dry cough, weight loss, and chest pain. Treatment involves long-term disease control with corticosteroids and short-term symptom management with oxygen therapy and over-the-counter medications. There is no cure, but supportive medicine can improve quality of life and increase life expectancy.

Desquamative interstitial pneumonia (DIP) is a type of noninfectious pneumonia known as idiopathic interstitial pneumonia. It can also be called alveolar macrophage pneumonia (AMP), the difference with DIP is that increases in macrophage cells are found. An increase in these cells triggers an immune system response. Interstitial inflammation occurs, which is a type of inflammation that causes tissue destruction.

Idiopathic interstitial pneumonia is classified as interstitial lung disease. Desquamative interstitial pneumonia is a rare form of this disease. Its main distinguishing symptom is an increase in macrophages. Macrophage cells are created from monocytes, which are types of white blood cells with the purpose of destroying foreign antigens and damaged cells. Due to the interstitial inflammation that occurs from pneumonia, the cells and tissues of the lungs are damaged. These damaged parts become a target for macrophages.

The causes of desquamative interstitial pneumonia are most commonly related to smoking. Most commonly, patients diagnosed with DIP are younger, averaging 36-46 years. Also, patients have usually been smoking heavily for several years.

Similar to idiopathic interstitial pneumonia, DIP symptoms include chronic dry cough, weight loss, and chest pain. Patients will also often experience shortness of breath and other breathing difficulties. Some patients also have a persistent low-grade fever.

Diagnosing desquamative interstitial pneumonia involves tests that allow doctors to view the inside of the lungs. Imaging tests can provide needed images of the lungs. These tests may include MRIs and ultrasounds. One of the definitive results that appears on the images is the large presence of macrophages which are brownish in color and occur in the air spaces of the alveoli in the lungs.

DIP treatment involves efforts for long-term disease control and short-term symptom management. Control of DIP is achieved by treatment with prescribed corticosteroids. Oxygen therapy can be used short-term to help with sporadic flare-ups causing breathing problems, or it can be used long-term when breathing has become permanently compromised. Over-the-counter medications can help relieve some symptoms, such as a dry cough.
There is no current cure for desquamative interstitial pneumonia. Supportive medicine can help patients reduce the frequency and severity of symptoms they experience. Symptom management can help improve quality of life and increase life expectancy, provided treatment is started quickly and continued. Without treatment, a patient with this lung disease cannot expect to be comfortable or to live beyond a few years.




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