Autoantibodies against constituents of neutrophils in the diagnosis and treatment of (isolated) subglottic stenosis.

Six patients had a subglottic stenosis either as a presenting symptom or as a manifestation of a systemic disease. All patients had in common the presence of circulating autoantibodies against constituents of neutrophils on indirect immunofluorescence. Cytoplasmic and perinuclear staining patterns were recognized. Such autoantibodies have been reported in Wegener's granulomatosis, microscopic polyarteritis, (idiopathic) glomerulonephritis, and Churg-Strauss syndrome. However, only one of the six patients fulfilled the criteria for these conditions. Because a positive test for autoantibodies against constituents of neutrophils is rare in other conditions and because other diseases had been excluded, we suggest that this places subglottic stenosis within the spectrum of necrotizing (granulomatous) vasculitis. The consequences for therapy are discussed.