Interstitial Lung Diseases Research

• An NHLBI-funded study discovered new mutations, or changes, in two genes associated with familial pulmonary fibrosis. This is a type of ILD that runs in families, and it causes life-threatening lung scarring in older adults. These findings helped advance our understanding of the causes of ILDs to help prevent lung scarring and improve quality of life.
• The NHLBI supports the Centers for Advanced Diagnostics and Experimental Therapeutics in Lung Diseases (CADET) program. This program stimulated the development of new treatments for lung diseases and sleep disorders. The first stage, CADET I, supported research on how lung diseases develop. The second stage, CADET II, funded research on new drugs. To learn more about new therapies for ILDs, visit NHLBI Supports CADET Researchers to Produce New Pulmonary Disease Drugs.
• NHLBI-funded researchers are helping find a new treatment for scleroderma-associated ILD. A medicine called oral cyclophosphamide (CYC) helps improve lung function in scleroderma-related ILD. However, patients can take this medicine for only a short term — up to a year. The researchers have tested another medicine, mycophenolate mofetil (MMF), and found that MMF is just as effective and easier for patients to tolerate without feeling sick. Also, research partially supported by the NHLBI has found that the medicine metformin, which is already approved to treat type 2 diabetes, has the potential to help treat lung fibrosis that gets worse over time.

Our Division of Lung Diseases and its Lung Biology and Disease Branch oversee much of the research on ILDs we fund.

Current research on lung biology and the causes of ILDs

• Older adults have a higher risk of some types of ILDs. The NHLBI supports research to find out how normal changes in our immune system due to aging can raise the risk of lung scarring. The researchers are also working to determine how age-related changes in the lungs can lead to ILDs.
• NHLBI-funded researchers are studying the role of the immune system and molecules called oxidants in lung scarring. The researchers are using animal models and donated lung tissue from people who have ILDs and asthma to find out how a protein called S-glutathionylation contributes to lung scarring. This research may lead to the development of specific antioxidants to prevent and treat ILDs.
• The NHLBI funds research to identify how specific cell types in the lungs are involved in lung scarring. The researchers are studying how the injury and death of cells in the lungs can trigger lung scarring and cause ILDs.

Find more NHLBI-funded studies on the causes of ILDs at the NIH RePORTER.

Current research on genetics and ILDs

• Some ILDs run in families. These conditions are caused by mutations, or changes in genes that control how our lungs develop or how our lungs respond to damage. The NHLBI supports research to identify genes that cause or raise the risk of a type of ILD called familial interstitial pneumonia. The researchers also aim to find out how mutations in these genes lead to lung damage.
• Idiopathic pulmonary fibrosis (IPF) is the most common type of ILD. NIH-funded researchers are working with the Pulmonary Fibrosis Foundation to identify genes that are linked to IPF and to understand the various types of IPF. The researchers are also working to find out whether a person’s genes determine whether a medicine called N-acetylcysteine is effective in treating IPF.

Find more NHLBI-funded studies on genetics and ILDs at the NIH RePORTER.

Current research on diagnosing and monitoring ILDs

• The NHLBI funds research to develop new methods to diagnose and monitor a type of ILD called hypersensitivity pneumonitis. NHLBI-supported researchers are focused on developing blood tests to monitor people who have hypersensitivity pneumonitis to identify which patients will develop serious complications from this type of ILD.
• To help better diagnose ILDs, NHLBI-funded researchers are using a new technique called high attenuation areas to improve the quality of images from computed tomography (CT) scans. The researchers will use this method to develop ways to predict which people who have mild lung scarring will go on to develop ILDs. This research will involve participants from the NHLBI-funded Multi-Ethnic Study of Atherosclerosis (MESA). The researchers will also establish a new study called the Families-At-risk for ILD (FAR-­ILD) study.
• NHLBI-funded researchers are developing new methods to use magnetic resonance imaging (MRI) technology to measure and monitor lung function in people who have IPF. This will help doctors and researchers find out whether new treatments for ILDs are effective in slowing down or stopping lung scarring. This research will involve both animal models and patients who have IPF.

Find more NHLBI-funded studies on diagnosing ILDs at the NIH RePORTER. 

Current research on treating ILDs

• Childhood interstitial lung disease (chILD) can be caused by mutations, or changes, in the genes that control how the body makes a substance called surfactant. Surfactant coats the inside of the lungs and helps the lungs work better. NHLBI-supported researchers are focused on using CRISPR-Cas9 gene editing technology to correct gene mutations that cause problems with surfactant production. This method of gene therapy could help treat life-threatening chILD.
• The NHLBI supports research to find new treatments to slow down or stop lung scarring in people who have ILDs. The researchers are working to identify specific proteins in the body that cause scar tissue to form in the lungs. They will then develop medicines to block these proteins. The researchers will also find out whether removing lung cells that usually form scar tissue can help prevent lung scarring from getting worse in people who have ILDs.

Find more NHLBI-funded studies on treating ILDs at the NIH RePORTER.

NHLBI in the Press

Learn more about NHLBI-supported research on treating ILDs: Researchers engineer cells to help reverse pulmonary fibrosis.

Researchers from the Laboratory of Applied Precision Omics, within the Pulmonary Branch of our Division of Intramural Research, are developing new methods to monitor and treat lung transplant rejection. Researchers from the Laboratory of Translational Research, also within the Pulmonary Branch, are focused on understanding the cause of an ILD called lymphangioleiomyomatosis (LAM).

Read more about these projects and ongoing clinical trials.

• The Molecular Atlas of Lung Development Program (LungMAP) is building a molecular map of the developing lungs in humans and mice. The program is helping advance lung research, in part through its web-based data resource, called BREATH, that allows users to access LungMAP data and findings. Learn more about LungMAP: NHLBI project breathes life into first in-depth atlas of the human lung.
• The Lung Tissue Research Consortium (LTRC) provides human lung tissues to qualified investigators for use in their research. The program enrolls patients who are planning to have lung surgery, collects blood and other clinical data from these donors, and stores donated tissue that otherwise would be discarded after the lung surgery. The LTRC provides tissue samples and data at no cost to approved investigators.
• The Pulmonary Trials Cooperative brings together patients, researchers, and healthcare professionals from more than 50 institutions, with a common goal of developing new treatments and testing current clinical care practices for ILDs and other lung diseases.
• The Biologic Specimen and Data Repository Information Coordinating Center (BioLINCC) centralizes and integrates biospecimens and clinical data that were once stored in separate repositories. Researchers can find and request available resources on BioLINCC’s secure website, which maximizes the value of these resources and advances heart, lung, blood, and sleep research.
• Our Trans-Omics for Precision Medicine (TOPMed) program includes participants who have ILDs, which may help us understand how genes contribute to differences in disease severity and how patients respond to treatment.
• Read about past NHLBI-funded workshops on promoting lung health in children and adults:
  • Defining and Promoting Pediatric Pulmonary Health (DAP3H)
  • NHLBI Workshop on Intersection between Aging Biology and Pathobiology of Lung Diseases