A team of investigators led by members of the University of Colorado School of Medicine faculty at CU Anschutz Medical Campus has identified a connection between mucus in the small airways and pulmonary fibrosis.
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“The overproduction of a lung mucin (MUC5B) has consistently been shown to be the strongest risk for the development of idiopathic pulmonary fibrosis (IPF) and most recently rheumatoid arthritis-interstitial lung disease,” said senior and corresponding author David Schwartz, MD, chair of the Department of Medicine at the CU School of Medicine.
The investigators hypothesized that the potential role for mucociliary dysfunction as a driver of IPF pathology is supported by unique gene expression signatures in IPF. The investigators found that a specific genetic characteristic, known as the MUC5B promoter variant rs35705950, which results in a marked increase production of mucus in the lung is the strongest genetic risk factor for IPF. They also found this is the strongest risk factor for rheumatoid arthritis-interstitial lung disease.
The findings suggest that targeting MUC5B in the terminal airways of patients with preclinical stages of interstitial lung disease represents a strategy to prevent the progression of preclinical pulmonary fibrosis.
“The findings in this manuscript provide a critical breakthrough in understanding the cause and potentially the treatment of IPF by demonstrating that excess mucus in the small airways can cause lung fibrosis, in part, by impairing the mechanism of lung clearance. In aggregate, these discoveries have provided the means to identify an at-risk population, diagnose the disease prior to the development of irreversible scarring, focus on a unique therapeutic target (MUC5B) and a specific location in the lung (distal airway), and create a novel pathway for therapeutic intervention for a disease that is currently incurable.”