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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*
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: Natasha.4.jordan@kcl.ac.uk

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.

Type
Main Articles
Copyright
Copyright © JLO (1984) Limited 2014 

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References

1Langford, CA, Sneller, MC, Hallahan, CW, Hoffman, GS, Kammerer, WA, Talar-Williams, C et al. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. Arthritis Rheum 1996;39:1754–60CrossRefGoogle ScholarPubMed
2Lebovics, RS, Hoffmann, GS, Leavitt, RY, Kerr, GS, Travis, WD, Kammerer, W et al. The management of subglottic stenosis in patients with Wegener's granulomatosis. Laryngoscope 1992;102:1341–5Google Scholar
3D'Cruz, DP, Baguley, E, Asherson, RA, Hughes, GR. Ear, nose, and throat symptoms in subacute Wegener's granulomatosis. BMJ 1989;299:419–22Google Scholar
4Ernst, A, Rafeq, S, Boiselle, P, Sung, A, Reddy, C, Michaud, G et al. Relapsing polychondritis and airway involvement. Chest 2009;135:1024–30CrossRefGoogle ScholarPubMed
5Solans-Laqué, R, Bosch-Gil, J, Canela, M, Lorente, J, Pallisa, E, Vilardell-Tarrés, M. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. Lupus 2008;17:832–6CrossRefGoogle ScholarPubMed
6Jennete, JC, Falk, RJ, Andrassy, K, Bacon, PA, Churg, J, Gross, WL et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187–92CrossRefGoogle Scholar
7Hunder, GG, Arend, WP, Bloch, DA, Calabrese, LH, Fauci, AS, Fries, JF et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum 1990;33:1065–7CrossRefGoogle ScholarPubMed
8Mukhtyar, C, Lee, R, Brown, D, Carruthers, D, Dasgupta, B, Dubey, S et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis 2009;68:1827–32Google Scholar
9Exley, AR, Bacon, PA, Luqmani, RA, Kitas, GD, Carruthers, DM, Moots, R. Examination of disease severity in systemic vasculitis from the novel perspective of damage using the vasculitis damage index (VDI). Br J Rheumatol 1998;37:5763CrossRefGoogle ScholarPubMed
10Myer, CM 3rd, O'Connor, DM, Cotton, RT. Proposed grading system for subglottic stenosis based on endotracheal tube sizes. Ann Otol Rhinol Laryngol 1994;103:319–23CrossRefGoogle ScholarPubMed
11Stegeman, CA, Tervaert, JW, de Jong, PE, Kallenberg, CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med 1996;335:1620Google Scholar
12Venning, MC, Arfeen, S, Bird, AG. Antibodies to neutrophil cytoplasmic antigen in systemic vasculitis. Lancet 1987;2:850CrossRefGoogle ScholarPubMed
13Lockwood, CM, Bakes, D, Jones, S, Whitaker, KB, Moss, DW, Savage, CO. Association of alkaline phosphatase with an autoantigen recognised by circulating anti-neutrophil antibodies in systemic vasculitis. Lancet 1987;1:716–20Google Scholar
14Langford, CA, Hoffman, GS. Rare diseases. 3: Wegener's granulomatosis. Thorax 1999;54:629–37Google Scholar
15Rees, JD, Lança, S, Marques, PV, Gómez-Puerta, JA, Moco, R, Oliveri, C et al. Prevalence of the antiphospholipid syndrome in primary systemic vasculitis. Ann Rheum Dis 2006;65:109–11Google Scholar
16Roediger, FC, Orloff, LA, Courey, MS. Adult subglottic stenosis: management with laser incisions and mitomycin-C. Laryngoscope 2008;118:1542–6Google Scholar
17Agrawal, N, Morrison, GA. Laryngeal cancer after topical mitomycin C application. J Laryngol Otol 2006;120:1075–6Google Scholar
18Schokkenbroek, AA, Franssen, CF, Dikkers, FG. Dilatation tracheoscopy for laryngeal and tracheal stenosis in patients with Wegener's granulomatosis. Eur Arch Otorhinolaryngol 2008;265:549–55CrossRefGoogle ScholarPubMed
19Hoffman, GS, Thomas-Golbanov, CK, Chan, J, Akst, LM, Eliachar, I. Treatment of subglottic stenosis, due to Wegener's granulomatosis, with intralesional corticosteroids and dilation. J Rheumatol 2003;30:1017–21Google ScholarPubMed
20van den Boogert, J, Hans Hoeve, LJ, Struijs, A, Hagenouw, RR, Bogers, AJ. Single-stage surgical repair of benign laryngotracheal stenosis in adults. Head Neck 2004;26:111–17Google Scholar