Reviewed by Gregory P. Cosgrove, , Jeffrey James Swigris, , Tristan J. Huie,
Interstitial lung disease (ILD) is a broad category of lung diseases that includes more than 130 disorders which are characterized by scarring (i.e. “fibrosis”) and/or inflammation of the lungs.

ILD accounts for 15 percent of the cases seen by pulmonologists (lung specialists).

In ILD, the tissue in the lungs becomes inflamed and/or scarred. The interstitium of the lung refers to the area in and around the small blood vessels and alveoli (air sacs). This is where the exchange of oxygen and carbon dioxide take place. Inflammation and scarring of the interstitium disrupts this tissue. This leads to a decrease in the ability of the lungs to extract oxygen from the air.

There are different types of interstitial lung disease that fall under the category of ILD. Some of the common ones are:

The progression of ILD varies from disease to disease and from person to person. It is important to determine the specific form of ILD in each person because what happens over time and the treatment may differ depending on the cause. Importantly, each person responds differently to treatment, so close monitoring during treatment is important.

Clinical Trials

A Linkage and Association Study in Pulmonary Fibrosis

The purpose of this study is to investigate inherited genetic factors that play a role in the development of familial pulmonary fibrosis and to identify a group of genes that predispose individuals to develop pulmonary fibrosis.  Finding the genes which play a role in pulmonary fibrosis is the first step at developing better methods for early diagnosis and improved treatment for pulmonary fibrosis. The overall hypothesis is that inherited genetic factors predispose individuals to develop pulmonary fibrosis.

The study requires the following:

  • For affected individuals, we confirm your diagnosis by reviewing your medical records. We also ask for a history questionnaire to be filled out and obtain a sample of blood or tissue for genetic studies.
  • Unaffected family members are screened with a questionnaire, and a blood sample is obtained for genetic studies.  Some family members may qualify to undergo high-resolution CT (HRCT) scan for the study.

To be eligible:

The only requirement for this study is to have at least 2 members of your family diagnosed with Idiopathic Pulmonary Fibrosis (IPF).  You may be included in this number.

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