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)