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

Published online by Cambridge University Press:  28 January 2009

J E Peters ,
A D Salama  and
P W Ind
J E Peters*
Affiliation:
Department of Respiratory Medicine, Hammersmith Hospital, London, UK
A D Salama
Affiliation:
Department of Renal Medicine, Hammersmith Hospital, London, UK
P W Ind
Affiliation:
Department of Respiratory Medicine, Hammersmith Hospital, London, UK
*
Address for correspondence: Dr James Peters, Department of Respiratory Medicine, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK. Fax: 0208 743 9733 E-mail: james.peters@imperial.nhs.uk
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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.

Keywords

Wegener's GranulomatosisTracheal StenosisVasculitisAntineutrophil Cytoplasmic AntibodiesGlomerulonephritis
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 12 , December 2009 , pp. 1375 - 1377
Copyright
Copyright © JLO (1984) Limited 2009

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