Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease.
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.
Peters
J E
JE
Department of Respiratory Medicine, Hammersmith Hospital, London, UK. james.peters@imperial.nhs.uk
Salama
A D
AD
Ind
P W
PW
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
D019268:Antibodies, Antineutrophil Cytoplasmic