Wegener’s Granulomatosis

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.
*Newer terminology is granulomatosis with polyangiitis (GPA)

See also: Wegener’s granulomatosis in the encyclopedia.