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Morbidity and mortality associated with subglottic laryngotracheal stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): a single-centre experience in the United Kingdom

Published online by Cambridge University Press:  02 September 2014

N P Jordan ,
H Verma ,
A Siddiqui ,
G A Morrison  and
D P D'Cruz
N P Jordan*
Affiliation:
Louise Coote Lupus Unit, St Thomas' Hospital, London, UK
H Verma
Affiliation:
Department of Radiology, St Thomas' Hospital, London, UK
A Siddiqui
Affiliation:
Department of Radiology, St Thomas' Hospital, London, UK
G A Morrison
Affiliation:
ENT Department, St Thomas' Hospital, London, UK
D P D'Cruz
Affiliation:
Louise Coote Lupus Unit, St Thomas' Hospital, London, UK
*
Address for correspondence: Dr Natasha Jordan, Louise Coote Lupus Unit, St Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, United Kingdom E-mail: [email protected]
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Abstract

Objectives:

We aimed to determine the prevalence of symptomatic subglottic laryngotracheal stenosis in patients with granulomatosis with polyangiitis (Wegener's granulomatosis); we also wanted to characterise the clinical outcomes and surgical interventions required, and the relapse rate in our cohort.

Methods:

We undertook a retrospective clinical review of all granulomatosis with polyangiitis patients with symptomatic subglottic laryngotracheal stenosis attending St Thomas' Hospital, London, United Kingdom.

Results:

Symptomatic subglottic laryngotracheal stenosis developed in 16 per cent of granulomatosis with polyangiitis patients attending our clinic. The median age of patients at diagnosis was 44 years (range: 34–81 years); 78 per cent of those presenting with subglottic laryngotracheal stenosis were women and 22 per cent were men. All patients were white; 67 per cent of patients were proteinase 3-antineutrophil cytoplasmic antibody-positive and 67 per cent developed relapsing disease requiring repeated surgical intervention. Subglottic laryngotracheal stenosis relapse was not associated with active systemic vasculitis elsewhere.

Conclusion:

Subglottic laryngotracheal stenosis is an uncommon but significant complication of granulomatosis with polyangiitis. Management of subglottic laryngotracheal stenosis requires a multi-disciplinary approach, with both rheumatological and otolaryngological expertise involved, given the relapsing nature of the disease.

Keywords

Wegener GranulomatosisAcquired Subglottic StenosisANCADiagnosisTherapy
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 128 , Issue 9 , September 2014 , pp. 831 - 837
Copyright
Copyright © JLO (1984) Limited 2014 

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