Interstitial Lung Disease
(Interstitial Pneumonia, Interstitial Pneumonitis)
Medical Author:
Melissa Conrad Stöppler, MD
Medical Editor:
William C. Shiel Jr., MD, FACP, FACR
What is interstitial lung disease?
Interstitial lung disease is a term used to refer to a particular type of
inflammation of the interstitium of the lungs. The interstitium is the tissue
that surrounds and separates the tiny air sacs (alveolae) in the lungs.
Interstitial lung disease involves an inflammation of this supportive tissue
between the air sacs rather than inflammation in the air sacs themselves.
Interstitial inflammation is typically a diffuse process that occurs all over
the lungs and is not confined to one location.
Sometimes the term "interstitial pneumonia" is used for certain forms of
interstitial lung disease. Technically, however, the term "pneumonia" refers to
inflammation of the lung tissue that is usually associated with infection.
Because interstitial lung disease has a number of causes (most of which are not
related to infection), the term "interstitial pneumonitis" is occasionally used
to refer to the inflammation that occurs in the absence of true infection.
What causes interstitial lung disease?
Interstitial lung disease is felt to be caused by a misdirected immune or
healing reaction to a number of factors, including:
Sometimes, the process of inflammation and scarring of the interstitial
tissues of the lungs develops in the absence of a known cause. When no cause can
be identified, this is referred to as idiopathic (unknown cause) interstitial
lung disease or idiopathic pulmonary fibrosis. Depending upon the location,
severity, and pattern of lung involvement, the idiopathic interstitial lung
diseases have been further subdivided into categories. Examples of different
types of idiopathic interstitial lung disease include:
- usual interstitial
pneumonitis (UIP),
- bronchiolitis
obliterans with organizing pneumonia (BOOP),
- lymphocytic interstitial pneumonitis (LIP), and
- desquamative interstitial
pneumonitis (DIP).
Next: What are the symptoms of interstitial lung disease? »
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