Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease

J Laryngol Otol. 2009 Dec;123(12):1375-7. doi: 10.1017/S002221510900454X. Epub 2009 Jan 28.


Objective: We report a patient with a 20-year history of apparently idiopathic airways stenoses, who presented with an antineutrophil cytoplasmic antibody (ANCA) associated, acute, systemic vasculitis with necrotising glomerulonephritis, subsequently diagnosed as Wegener's granulomatosis.

Methods: We present a case report and a review of the world literature on airway stenosis in Wegener's granulomatosis.

Results: To our knowledge, this is the first report of Wegener's granulomatosis manifesting as local airway disease for such a prolonged period, before transforming into a systemic vasculitis.

Conclusions: This case highlights the need for physicians to be alert to the possibility of Wegener's granulomatosis as a cause of apparently idiopathic airway stenosis, and to be aware that systemic disease may occur in very long-standing, limited Wegener's granulomatosis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / analysis
  • Diagnosis, Differential
  • Female
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / diagnosis
  • Humans
  • Middle Aged
  • Systemic Vasculitis / etiology*
  • Time Factors
  • Tracheal Stenosis / complications*
  • Treatment Outcome


  • Antibodies, Antineutrophil Cytoplasmic