Source
Department of Respiratory Medicine, Hammersmith Hospital, London, UK. james.peters@imperial.nhs.uk
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.