A rare inflammatory disease of small arteries and veins (vasculitis) that classically involves vessels supplying the tissues of the lungs, nasal passages (sinuses), and kidneys. At our practice, we see a large population of individuals with what we believe to be a forme fruste (incomplete expression) of Wegener’s granulomatosis* causing subglottic and tracheal stenosis.
*Newer terminology is granulomatosis with polyangiitis (GPA)
Subglottic Stenosis, Due to Wegener’s Granulomatosis
Airway Stenosis Caused By Wegener’s Granulomatosis, Before and After Dilations
Vascular Manifestations of Wegener’s-related Septum Changes, and Subglottic Stenosis Indistinguishable from Forme Fruste Wegener’s
Wegener’s Granulomatosis*: Forme Fruste (incomplete expression)
Wegener’s granulomatosis is a rare autoimmune disorder in which blood vessels become inflamed. The inflammation causes swelling and scarring. Three typical organs attacked are sinus/nasal cavities, lungs, and kidneys.
In the forme fruste variant, it is mostly an inflammatory stenosis (narrowing) of the area below the vocal folds, and also the trachea. A person becomes short of breath and begins to make harsh breathing sounds due to the narrowed passageway. This is an example of one means of management: dilation of the narrowed area during a very brief general anesthetic in an outpatient operating room.