Forme fruste Wegener’s granulomatosis

An incomplete or frustrated form (forme fruste) of Wegener’s granulomatosis,* which we believe to be the cause behind some cases of inflammatory subglottic or tracheal stenosis. Unlike full-fledged Wegener’s, this forme fruste variant may or may not necessarily involve the sinus and nasal cavities, and in the author’s caseload of about 60 patients, it has not ever progressed to involve the lungs and kidneys. Such patients can go for years with only the need for intermittent dilation of the subglottic or tracheal narrowing. This disorder may be the same as what some call “idiopathic subglottic stenosis,” for which some have recommended cricotracheal resection and reanastomosis as treatment.

*Newer terminology is granulomatosis with polyangiitis (GPA)



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 cords, 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.