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1 results

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

  • J E Peters, A D Salama, P W Ind
  • Journal:
    The Journal of Laryngology & Otology / Volume 123 / Issue 12 / December 2009
    Published online by Cambridge University Press:
    28 January 2009, pp. 1375-1377
    Print publication:
    December 2009
  • 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.