Study seeks to expand treatment options for rare airway disease

From the Fall 2019 edition of Vanderbilt Medicine Magazine

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Armed with $1.2 million in funding from the National Heart, Lung and Blood Institute (NHLBI), researchers at Vanderbilt University Medical Center are searching to understand the cause of a rare airway disease in hopes of developing better treatments.

Idiopathic subglottic stenosis (iSGS) is an unexplained narrowing of the windpipe just below the vocal cords. The disease is progressive, slowly affecting the patient’s ability to breathe, and can be fatal due to airway obstruction if left untreated.

Affecting fewer than one in 200,000 people, the disease occurs almost exclusively in adult, Caucasian women, with most patients presenting in their 40s or 50s.

“In taking care of these patients, it has always struck me that they appeared so similar,” said Alexander Gelbard, MD, associate professor of Otolaryngology and co-director of the Vanderbilt Complex Airway Center (AeroVU). “The tightness of the demographics makes me believe there is a common thread driving this disease that we just don’t yet understand.”

Although treatments for iSGS exist, they’re invasive, costly for the patient and don’t always prevent the condition from recurring.

The new five-year study will dive deeper into the immune system’s response at the site of the fibrosis, or scarring, that takes place in the area below the patients’ vocal cords to answer questions about the source of the disease, what makes it better or worse and why it often recurs following surgical treatment. n  Kelsey Herbers