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Idiopathic Infiltrative Lung Diseases

Overview :

The body produces inflammatory cells in response to a variety of conditions, including a number of different diseases, pollutants, certain infections, exposure to organic dust or toxic fumes and vapors, and various drugs and poisons. When white blood cells and tissue fluid rich in protein collect in the small air sacs of the lungs, or alveoli, the sacs become inflamed (alveolitis). In time, the fluid may solidify and cause scar formation that replaces the normal lung tissue. This process is known as pulmonary fibrosis. In about half of all patients, no specific cause is ever found; they are said to have idiopathic pulmonary fibrosis.

Some patients have special types of interstitial lung disease that may occur in certain types of patients, or feature typical pathological changes when a sample of lung tissue is examined under a microscope. They include:

  • Usual interstitial pneumonitis. Disease occurs in a patchy form throughout the lungs. Parts of the lungs can appear normal while others have dense scar tissue and lung cysts, often the end result of pulmonary fibrosis. This disease progresses quite slowly. Both children and adults may be affected.
  • Desquamative interstitial pneumonitis. Similarappearing lesions are present throughout the lungs. Both inflammatory cells and cells that have separated from the air sac linings ("desquamated") are present. Some researchers believe this is an early form of usual interstitial pneumonitis.
  • Lymphocytic interstitial pneumonitis. Most of the cells infiltrating the lungs are the type of white blood cells called lymphocytes. Both the breathing tubes (bronchi) and blood vessels of the lungs become thickened. In children, this condition tends to occur when the immune system is not operating properly as occurs with Acquired Immune Deficiency Syndrome (AIDS).

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