title: Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease.
abstract: 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.
authors.affiliation: Department of Respiratory Medicine, Hammersmith Hospital, London, UK. james.peters@imperial.nhs.uk
authors.forename: J E
authors.initials: JE
authors.lastname: Peters
authors.forename: A D
authors.initials: AD
authors.lastname: Salama
authors.forename: P W
authors.initials: PW
authors.lastname: Ind
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008875:Middle Aged; D056647:Systemic Vasculitis / Q000209:etiology*; D013997:Time Factors; D014135:Tracheal Stenosis / Q000150:complications*; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic