Idiopathic Pulmonary Fibrosis Research

As part of its broader commitment to research on lung diseases, the NHLBI leads and supports research and programs on idiopathic pulmonary fibrosis (IPF). NHLBI-supported research has shown that certain treatments for IPF can be harmful. Our research has also helped identify genes that are linked to IPF. Current studies are focused on finding new treatments for IPF and improving our understanding of how our genes and other factors raise the risk of this lung condition.

NHLBI research that really made a difference

NHLBI research that really made a difference
  • The NHLBI formed the IPF Clinical Research Network (IPFnet) in 2005 to support research on IPF treatment. One study, PANTHER-IPF, found that a commonly used treatment for people who have IPF could be harmful. The triple-medicine treatment included prednisone, azathioprine, and N-acetylcysteine. Guidelines for IPF now recommend against the three-medicine treatment, which is a change in clinical practice that has likely prevented unnecessary harm to other patients receiving care for IPF. Read more about this trial: Commonly used three-drug regimen for idiopathic pulmonary fibrosis found harmful.
  • In 2012, the NHLBI organized the Strategic Planning for Idiopathic Pulmonary Fibrosis workshop to draft a strategic plan for IPF research. The workshop brought together leaders in IPF research and representatives from the pharmaceutical industry, regulatory bodies, and patient advocacy groups. Workshop participants recommended that IPF researchers focus on collaborative research studies, studies that compare healthy lung tissue and lung tissue from people who have IPF, and studies to understand how genes and the environment can raise the risk of IPF. These recommendations have served as a roadmap for IPF research.
  • NHLBI-supported research confirmed the association between a gene called MUC5B and IPF that greatly increases the risk of pulmonary fibrosis. The NHLBI currently funds research to find out more about how MUC5B raises the risk of IPF. Read more about this research: Genes and Transcripts that Interact with MUC5B in Pulmonary Fibrosis.

Current research funded by the NHLBI

Current research funded by the NHLBI

Our Division of Lung Diseases and its Lung Biology and Disease Branch supports research on lung biology and lung diseases. Our Granulomatous and Interstitial Lung Disease Program supports laboratory research and clinical studies on the causes, risk factors, diagnosis, and treatment of interstitial lung diseases, including IPF.

Current research on the risk factors for IPF

  • The NHLBI supports research to better understand the risk factors for IPF. Researchers are developing new laboratory models of IPF to study what makes damaged lungs form either healthy tissue or scar tissue after an injury. Understanding the causes of IPF will help researchers develop new treatments for this condition.
  • NHLBI-funded researchers are looking at how our genes and environment can raise the risk of pulmonary fibrosis. The scientists are collecting information and samples from hundreds of healthy people, people who have pulmonary fibrosis, and people who have a high risk of the disease. This data will help identify new risk factors and help us better understand how lung scarring happens in people with pulmonary fibrosis.
  • The NHLBI supports research to develop robust screening methods for IPF. Early treatment for IPF can help slow down lung damage. However, it can be challenging for doctors to diagnose IPF at its earliest stages. Researchers are looking for common factors that help predict which people who are at high risk of IPF actually end up developing this disease. The new diagnostic tools will help doctors monitor people who are most likely to develop lung scarring and to identify the first signs of IPF.

Find more NHLBI-funded studies on IPF risk factors at NIH RePORTER. 

Current research on new treatments for IPF

  • The NHLBI funds research to develop new medicines to treat IPF by targeting problems with how the body makes certain proteins. In people who have IPF, problems with how the lungs make proteins can cause lung scarring. New medicines will block the molecules that cause these problems with protein production to protect the lungs from further damage.
  • NHLBI-funded researchers are conducting clinical trials to test the safety and effectiveness of medicines to treat IPF. The researchers are also using data on how patients respond to the new medicines to better understand the processes that cause lung scarring. Additionally, the researchers are studying how the immune system and our genes affect a person’s response to IPF treatment.
  • The NHLBI supports the Prospective tReatment EffiCacy in IPF uSIng genOtype for Nac Selection (PRECISIONS) trial to improve treatment for IPF. This study is looking at whether differences in a person’s genes affect their response to a medicine called N-Acetylcysteine (NAC). The researchers are also looking for markers that can help doctors tell the difference between IPF and other interstitial lung diseases.

Find more NHLBI-funded studies on treatment for IPF at NIH RePORTER. 

Current research on lung biology

  • The NHLBI funds research to find out how normal changes in the lungs as people get older can affect the risk of IPF. As we age, the cells in our lungs might not function as well as they used to, and this raises the risk of lung disease. Researchers will find out whether the same processes that raise the risk of lung disease in older adults contribute to lung disease in other age groups.
  • NHLBI-supported researchers are looking at how stem cells in lungs help the lungs heal after an injury. Problems with lung healing might cause scar tissue to form instead of healthy lung tissue, and this can cause IPF.
  • The NHLBI supports research on how a type of tissue called the alveolar epithelium contributes to lung health. The alveolar epithelium is a thin layer of tissue that surrounds and protects the lungs’ air sacs, or alveoli. Researchers are looking at how this tissue helps repair lung damage and its role in scar tissue formation.

Find more NHLBI-funded studies on IPF and lung biology at NIH RePORTER. 

IPF research labs at the NHLBI

IPF research labs at the NHLBI

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. 

Related IPF programs

Related IPF programs

Explore more NHLBI research on IPF

The sections above provide you with the highlights of NHLBI-supported research on IPF. You can explore the full list of NHLBI-funded studies on the NIH RePORTER.

To find more studies:

  • Type your search words into the Quick Search box and press enter. 
  • Check Active Projects if you want current research.
  • Select the Agencies arrow, then the NIH arrow, then check NHLBI.

If you want to sort the projects by budget size from the biggest to the smallest click on the FY Total Cost by IC column heading.

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