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
- 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
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.
Related IPF programs
- The NHLBI has led several recent workshops on promoting lung health in children and adults. The Defining and Promoting Pediatric Pulmonary Health (DAP3H) workshop in 2021 explored current and future research on pediatric lung and sleep health. Also in 2021, the NHLBI held the How Can We Use Bioengineering Approaches to (Re)Build a Lung? workshop to evaluate current models of human lung biology and identify research priorities.
- 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 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 Biologic Specimen and Data Repository Information Coordinating Center (BioLINCC) provides a centralized system to manage biospecimens and clinical data that were once stored in separate repositories. Through BioLINCC, researchers can access data on several lung diseases, including IPF. Researchers can find and request available resources on BioLINCC’s secure website.
- 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 IPF and other lung diseases.
- Our Trans-Omics for Precision Medicine (TOPMed) program includes participants who have IPF, which may help us understand how genes contribute to differences in disease severity and how patients respond to treatment.