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Lung fibroblasts under microscope

Why does idiopathic pulmonary fibrosis progress faster?

Environmental Health LHI

Researchers from the Institute of Lung Health and Immunity investigated the role of an age-related phenomenon in the blood-forming system in lung fibrosis.

The study, published in JCI Insight, was led by group leader Isis Fernandez and first author Lori Asarian. It investigates the role of clonal hematopoiesis, an age-related process in the blood-forming system that has increasingly been linked to chronic diseases.

Idiopathic pulmonary fibrosis (IPF) is a severe and currently incurable lung disease characterized by progressive scarring of the lungs. Disease progression varies widely between patients, making it difficult to predict outcomes and tailor treatment strategies. Understanding the factors that drive rapid progression remains one of the most important challenges in the field.

Analyzing samples from patients with IPF, the research team found evidence of clonal hematopoiesis in 38 percent of cases. Patients carrying these mutations were significantly more likely to experience rapid disease progression compared with patients without the mutations. They also showed a substantially greater decline in lung function over a 12-month period.

First author Lori Asarian would like to thank all patients who participated in the study: “Their contribution was essential to uncover this connection that may have important implications for patients with IPF.”

Clonal hematopoiesis - a blood-based indicator of IPF progression?

The findings give new insights into how age-related changes in the hematopoietic system may influence lung fibrosis. "The study highlights the importance of looking beyond the lung itself to better understand disease mechanisms," says Isis Fernandez, group leader at LHI. "Our findings strengthen the link between age-related blood cell changes and pulmonary fibrosis and may help improve patient stratification in the future."

The study further reinforces the Institute of Lung Health and Immunity's leading expertise in pulmonary fibrosis research. Through close collaboration with clinical partners, LHI researchers are working to better understand the biological mechanisms underlying this devastating disease and to identify biomarkers that could support earlier intervention and more personalized treatment approaches.

Publication:

Asarian L. et al. Clonal hematopoiesis is associated with disease progression in idiopathic pulmonary fibrosis. JCI Insight. 2026

Read the full paper:  https://insight.jci.org/articles/view/198458