Hostname: page-component-586b7cd67f-t7fkt Total loading time: 0 Render date: 2024-11-28T12:09:14.379Z Has data issue: false hasContentIssue false
  • English
  • Français

Presentation and initial assessment of ENT problems in patients with granulomatosis with polyangiitis (Wegener's)

Published online by Cambridge University Press:  22 July 2014

M Martinez Del Pero ,
D McKiernan  and
P Jani
M Martinez Del Pero*
Affiliation:
Department of Otolaryngology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, UK
D McKiernan
Affiliation:
Department of Otolaryngology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, UK
P Jani
Affiliation:
Department of Otolaryngology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, UK
*
Address for correspondence: Mr M Martinez Del Pero, ENT Department, Box 48, Addenbrooke's Hospital, Hills Road, Cambridge CB2 0QQ, UK E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) is a primary systemic vasculitis that affects medium to small sized vessels throughout the body. It often presents with symptoms and signs involving the ear, nose, and head and neck area.

Objective:

To highlight salient features of ENT-related issues in granulomatosis with polyangiitis, and raise awareness of the condition.

Methods:

A case report of a patient with limited disease and an insidious onset is presented, along with a review of the current literature. In addition, basic initial management is described.

Results:

Eighty-six publications were used to describe salient features of ENT-related issues in granulomatosis with polyangiitis.

Conclusion:

The time to diagnosis has not reduced significantly in the last 10 years in the UK. A high index of suspicion is required for an earlier diagnosis of granulomatosis with polyangiitis.

Keywords

Wegener GranulomatosisVasculitisAntibodies, Antineutrophil CytoplasmicDiagnosis
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 128 , Issue 8 , August 2014 , pp. 730 - 737
Copyright
Copyright © JLO (1984) Limited 2014 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Wegener, F. On generalised septic vessel diseases. By Wegener, Friedrich, 1937 (translation). Thorax 1987;42:918–19Google Scholar
2Wegener, F. Wegener's granulomatosis. Thoughts and observations of a pathologist. Eur Arch Otorhinolaryngol 1990;247:133–42Google Scholar
3Falk, RJ, Gross, WL, Guillevin, L, Hoffman, G, Jayne, DR, Jennette, JC et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Ann Rheum Dis 2011;70:704Google Scholar
4Leavitt, RY, Fauci, AS, Bloch, DA, Michel, BA, Hunder, GG, Arend, WP et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101–7Google Scholar
5Jennette, 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 ScholarPubMed
6Watts, R, Lane, S, Hanslik, T, Hauser, T, Hellmich, B, Koldingsnes, W et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007;66:222–7Google Scholar
7Falk, RJ, Jennette, JC. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med 1988;318:1651–7Google Scholar
8Jennette, JC, Hoidal, JR, Falk, RJ. Specificity of anti-neutrophil cytoplasmic autoantibodies for proteinase 3. Blood 1990;75:2263–4Google Scholar
9Sinico, RA, Di Toma, L, Maggiore, U, Bottero, P, Radice, A, Tosoni, C et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005;52:2926–35Google Scholar
10Guillevin, L, Durand-Gasselin, B, Cevallos, R, Gayraud, M, Lhote, F, Callard, P et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999;42:421–30Google Scholar
11Lanham, JG, Elkon, KB, Pusey, CD, Hughes, GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine 1984;63:65–81Google Scholar
12Drinias, V, Florentzson, R. Facial palsy and Wegener's granulomatosis. Am J Otolaryngol 2004;25:208–12Google Scholar
13D'Cruz, DP, Baguley, E, Asherson, RA, Hughes, GR. Ear, nose, and throat symptoms in subacute Wegener's granulomatosis. BMJ 1989;299:419–22Google Scholar
14Carrington, CB, Liebow, A. Limited forms of angiitis and granulomatosis of Wegener's type. Am J Med 1966;41:497–527Google Scholar
15Devaney, KO, Ferlito, A, Hunter, BC, Devaney, SL, Rinaldo, A. Wegener's granulomatosis of the head and neck. Ann Otol Rhinol Laryngol 1998;107:439–45CrossRefGoogle Scholar
16McDonald, TJ, DeRemee, RA, Kern, EB, Harrison, EG Jr.Nasal manifestations of Wegener's granulomatosis. Laryngoscope 1974;84:2101–12Google Scholar
17Takala, JH, Kautiainen, H, Malmberg, H, Leirisalo-Repo, M. Wegener's granulomatosis in Finland in 1981-2000: clinical presentation and diagnostic delay. Scand J Rheumatol 2008;37:435–8Google Scholar
18Srouji, IA, Andrews, P, Edwards, C, Lund, VJ. Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects. J Laryngol Otol 2007;121:653–8Google Scholar
19Lane, SE, Watts, RA, Shepstone, L, Scott, DG. Primary systemic vasculitis: clinical features and mortality. QJM 2005;98:97–111Google Scholar
20Hoffman, GS, Kerr, GS, Leavitt, RY, Hallahan, CW, Lebovics, RS, Travis, WD et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488–98CrossRefGoogle ScholarPubMed
21Bligny, D, Mahr, A, Toumelin, PL, Mouthon, L, Guillevin, L. Predicting mortality in systemic Wegener's granulomatosis: a survival analysis based on 93 patients. Arthritis Rheum 2004;51:83–91CrossRefGoogle ScholarPubMed
22Walsh, M, Flossman, O, Berden, A, Westman, K, Manns, B, Jayne, D. The association between relapses of ANCA-associated vasculitis and the composite endpoint of end-stage renal disease or death. APMIS 2009;117:143–4Google Scholar
23Sproson, E, Jones, N, Al-Deiri, M, Lanyon, P. Lessons learnt in the management of Wegener's granulomatosis: long-term follow-up of 60 patients. Rhinology 2007;45:63–7Google ScholarPubMed
24Herlyn, K, Seo, P, Hellmich, B, Reimer, J, Merkel, PA. Patient-reported outcome assessment in primary systemic vasculitis with a new instrument provides a unique perspective. APMIS 2009;117(suppl 127):149Google Scholar
25Illum, P, Thorling, K. Otological manifestations of Wegener's granulomatosis. Laryngoscope 1982;92:801–4CrossRefGoogle ScholarPubMed
26Takagi, D, Nakamaru, Y, Maguchi, S, Furuta, Y, Fukuda, S. Otologic manifestations of Wegener's granulomatosis. Laryngoscope 2002;112:1684–90Google Scholar
27Kornblut, AD, Wolff, SM, Fauci, AS. Ear disease in patients with Wegener's granulomatosis. Laryngoscope 1982;92:713–17CrossRefGoogle ScholarPubMed
28Kempf, HG. Ear involvement in Wegener's granulomatosis. Clin Otolaryngol Allied Sci 1989;14:451–6Google Scholar
29Reinhold-Keller, E, Beuge, N, Latza, U, de Groot, K, Rudert, H, Nolle, B et al. An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 2000;43:1021–32Google Scholar
30Fauci, AS, Haynes, BF, Katz, P, Wolff, SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98:76–85CrossRefGoogle Scholar
31Ferri, E, Armato, E, Capuzzo, P, Cavaleri, S, Ianniello, F. Early diagnosis of Wegener's granulomatosis presenting with bilateral facial paralysis and bilateral serous otitis media. Auris Nasus Larynx 2007;34:379–82Google Scholar
32Pereira, D, Ayres do Amaral, J, Mansur Szajubok, J, Loduca Lima, S, Chahade, WH. Otorhinolaryngologic manifestations of autoimmune rheumatic diseases. Rev Bras Reumatol 2006;46:118–25Google Scholar
33Burlacoff, SG, Wong, FS. Wegener's granulomatosis. The great masquerade: a clinical presentation and literature review. J Otolaryngol 1993;22:94–105Google Scholar
34Bucolo, S, Torre, V, Montemagno, A, Beatrice, F. Wegener's granulomatosis presenting with otologic and neurologic symptoms: clinical and pathological correlations. J Oral Pathol Med 2003;32:438–40Google Scholar
35Calonius, IH, Christensen, CK. Hearing impairment and facial palsy as initial signs of Wegener's granulomatosis. J Laryngol Otol 1980;94:649–57Google Scholar
36Clements, MR, Mistry, CD, Keith, AO, Ramsden, RT. Recovery from sensorineural deafness in Wegener's granulomatosis. J Laryngol Otol 1989;103:515–18Google Scholar
37Ferlito, A, Devaney, KO, Anniko, M, Arnold, W, Rinaldo, A. Otological Wegener's granulomatosis at the time of initial presentation: a potential diagnostic dilemma. Acta Otolaryngol 2003;123:675–7CrossRefGoogle ScholarPubMed
38Friedmann, I, Bauer, F. Wegener's granulomatosis causing deafness. J Laryngol Otol 1973;87:449–64CrossRefGoogle ScholarPubMed
39Helmberger, RC, Mancuso, AA. Wegener granulomatosis of the eustachian tube and skull base mimicking a malignant tumor. AJNR Am J Neuroradiol 1996;17:1785–90Google Scholar
40Hern, JD, Hollis, LJ, Mochloulis, G, Montgomery, PQ, Tolley, NS. Early diagnosis of Wegener's granulomatosis presenting with facial nerve palsy. J Laryngol Otol 1996;110:459–61Google Scholar
41Nikolaou, AC, Vlachtsis, KC, Daniilidis, MA, Petridis, DG, Daniilidis, IC. Wegener's granulomatosis presenting with bilateral facial nerve palsy. Eur Arch Otorhinolaryngol 2001;258:198–202CrossRefGoogle ScholarPubMed
42Atula, T, Honkanen, V, Tarkkanen, J, Jero, J. Otitis media as a sign of Wegener's granulomatosis in childhood. Acta Otolaryngol Suppl 2000;543:48–50Google ScholarPubMed
43Murty, GE. Wegener's granulomatosis: otorhinolaryngological manifestations. Clin Otolaryngol Allied Sci 1990;15:385–93CrossRefGoogle ScholarPubMed
44Bakthavachalam, S, Driver, MS, Cox, C, Spiegel, JH, Grundfast, KM, Merkel, PA. Hearing loss in Wegener's granulomatosis. Otol Neurotol 2004;25:833–7Google Scholar
45Jennings, CR, Jones, NS, Dugar, J, Powell, RJ, Lowe, J. Wegener's granulomatosis--a review of diagnosis and treatment in 53 subjects. Rhinology 1998;36:188–91Google ScholarPubMed
46Kornblut, AD, Wolff, SM, deFries, HO, Fauci, AS. Wegener's granulomatosis. Laryngoscope 1980;90:1453–65CrossRefGoogle ScholarPubMed
47Stone, JH. Limited versus severe Wegener's granulomatosis: baseline data on patients in the Wegener's granulomatosis etanercept trial. Arthritis Rheum 2003;48:2299–309Google Scholar
48Yucel, EA, Keles, N, Ozturk, AS, Solmaz, MA, Deger, K. Wegener's granulomatosis presenting in the sinus and orbit. Otolaryngol Head Neck Surg 2002;127:349–51Google Scholar
49Kornblut, AD, Wolff, SM, deFries, HO, Fauci, AS. Wegener's granulomatosis. Otolaryngol Clin North Am 1982;15:673–83Google Scholar
50Belostostky, VM, Shah, V, Dillon, MJ. Clinical features in 17 paediatric patients with Wegener granulomatosis. Pediatr Nephrol 2002;17:754–61Google Scholar
51Rottem, M, Fauci, AS, Hallahan, CW, Kerr, GS, Lebovics, R, Leavitt, RY et al. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr 1993;122:26–31CrossRefGoogle ScholarPubMed
52Cohen, PS, Meltzer, JA. Strawberry gums. A sign of Wegener's granulomatosis. JAMA 1981;246:2610–11Google Scholar
53Berge, S, Niederhagen, B, von Lindern, JJ, Appel, T, Reich, RH. Salivary gland involvement as an initial presentation of Wegener's disease. A case report. Int J Oral Maxillofac Surg 2000;29:450–2Google Scholar
54Singh, S, Kaura, D, Kumari, S, Gupta, D, Dey, P, Suri, S. Wegener's presenting as submandibular swelling. Sarcoidosis Vasc Diffuse Lung Dis 1997;14:81–2Google ScholarPubMed
55Garcia, PC, Amor, DJC, Gonzalez, GMA. Unilateral submandibular swelling as unique presentation of Wegener's granulomatosis. Rheumatology (Oxford) 2001;40:953–4Google Scholar
56Bulbul, Y, Ozlu, T, Oztuna, F. Wegener's granulomatosis with parotid gland involvement and pneumothorax. Med Princ Pract 2003;12:133–7Google Scholar
57Liu, SY, Vlantis, AC, Lee, WC. Bilateral parotid and submandibular gland enlargement: rare features of Wegener's granulomatosis. J Laryngol Otol 2003;117:148–50Google Scholar
58Hoare, TJ, Jayne, D, Rhys Evans, P, Croft, CB, Howard, DJ. Wegener's granulomatosis, subglottic stenosis and antineutrophil cytoplasm antibodies. J Laryngol Otol 1989;103:1187–91CrossRefGoogle ScholarPubMed
59Di Comite, G, Bonavida, G, Bozzolo, E, Bianchi, S, Ciboddo, G, Tresoldi, M et al. Wegener's granulomatosis: an analysis of 50 patients [in Italian]. Reumatismo 2005;57:187–92Google Scholar
60Harper, SL, Letko, E, Samson, CM, Zafirakis, P, Sangwan, V, Nguyen, Q et al. Wegener's granulomatosis: the relationship between ocular and systemic disease. J Rheumatol 2001;28:1025–32Google Scholar
61Robin, JB, Schanzlin, DJ, Meisler, DM, deLuise, VP, Clough, JD. Ocular involvement in the respiratory vasculitides. Surv Ophthalmol 1985;30:127–40Google Scholar
62Dorr, J, Elitok, S, Dieste, FJ, Vogel, HP, Schneider, W, Wuerfel, J et al. Treatment-resistant chronic headaches and focal pachymeningitis in a 46-year-old man: a rare presentation of Wegener's granulomatosis. Lancet Neurol 2008;7:368–72Google Scholar
63Fam, AG, Lavine, E, Lee, L, Perez-Ordonez, B, Goyal, M. Cranial pachymeningitis: an unusual manifestation of Wegener's granulomatosis. J Rheumatol 2003;30:2070–4Google ScholarPubMed
64Aries, PM, Reinhold-Keller, E, Gross, WL. Another look at Wegener's granulomatosis-associated pachymeningitis. J Rheumatol 2004;31:825; author reply 825–6Google Scholar
65Haynes, BF, Fauci, AS. Diabetes insipidus associated with Wegener's granulomatosis successfully treated with cyclophosphamide. N Engl J Med 1978;299:764CrossRefGoogle ScholarPubMed
66Yong, TY, Li, JY, Amato, L, Mahadevan, K, Phillips, PJ, Coates, PS et al. Pituitary involvement in Wegener's granulomatosis. Pituitary 2008;11:77–84Google Scholar
67Lohr, KM, Ryan, LM, Toohill, RJ, Anderson, T. Anterior pituitary involvement in Wegener's granulomatosis. J Rheumatol 1988;15:855–7Google Scholar
68McLaren, JS, McRorie, ER, Luqmani, RA. Diagnosis and assessment of systemic vasculitis. Clin Exp Rheumatol 2002;20:854–62Google Scholar
69Anderson, G, Coles, ET, Crane, M, Douglas, AC, Gibbs, AR, Geddes, DM et al. Wegener'sgranuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. Q J Med 1992;83:427–38Google ScholarPubMed
70van der Woude, FJ, Rasmussen, N, Lobatto, S, Wiik, A, Permin, H, van Es, LA, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet 1985;1:425–9Google Scholar
71Savige, J, Dimech, W, Fritzler, M, Goeken, J, Hagen, EC, Jennette, JC et al. Addendum to the International Consensus Statement on testing and reporting of antineutrophil cytoplasmic antibodies. Quality control guidelines, comments, and recommendations for testing in other autoimmune diseases. Am J Clin Pathol 2003;120:312–18Google Scholar
72Andrassy, K, Rasmussen, N. Treatment of granulomatous disorders of the nose and paranasal sinuses. Rhinology 1989;27:221–30Google Scholar
73Raynaud, P, Garrel, R, Rigau, V, Poizat, F, Vic, P, Cartier, C et al. How can the diagnostic value of head and neck biopsies be increased in Wegener's granulomatosis: a clinicopathologic study of 49 biopsies in 21 patients [in French]. Ann Pathol 2005;25:87–93Google Scholar
74Devaney, KO, Travis, WD, Hoffman, G, Leavitt, R, Lebovics, R, Fauci, AS. Interpretation of head and neck biopsies in Wegener's granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol 1990;14:555–64Google Scholar
75Rasmussen, N. Ear, nose and throat manifestations in c-ANCA-positive vasculitides. Diagnostic, therapeutic and pathogenetic implications. Ann Med Interne (Paris) 1992;143:401–4Google Scholar
76Rasmussen, N, Petersen, J, Jensen, H, Andersen, V. Histopathological findings in biopsies from patients with Wegener's granulomatosis. APMIS Suppl 1990;19:15–16Google Scholar
77Del Buono, EA, Flint, A. Diagnostic usefulness of nasal biopsy in Wegener's granulomatosis. Hum Pathol 1991;22:107–10Google Scholar
78Diamantopoulos, II, Jones, NS. The investigation of nasal septal perforations and ulcers. J Laryngol Otol 2001;115:541–4Google Scholar
79Benoudiba, F, Marsot, DK, Hadj, RM, Cabanne, J, Bobin, S, Lasjaunias, P. Sinonasal Wegener's granulomatosis: CT characteristics. Neuroradiology 2003;45:95–9Google Scholar
80Allen, SD, Harvey, CJ. Imaging of Wegener's granulomatosis. Br J Radiol 2007;80:757–65Google Scholar
81Silvera, S, Vignaux, O, Legmann, P. Sinonasal and cerebral imaging findings in Wegener's granulomatosis [in French]. Presse Med 2007;36:913–21Google Scholar
82Lohrmann, C, Uhl, M, Warnatz, K, Kotter, E, Ghanem, N, Langer, M. Sinonasal computed tomography in patients with Wegener's granulomatosis. J Comput Assist Tomogr 2006;30:122–5Google Scholar
83Lloyd, G, Lund, VJ, Beale, T, Howard, D. Rhinologic changes in Wegener's granulomatosis. J Laryngol Otol 2002;116:565–9Google Scholar
84Quint, LE, Whyte, RI, Kazerooni, EA, Martinez, FJ, Cascade, PN, Lynch, JP 3rd et al. Stenosis of the central airways: evaluation by using helical CT with multiplanar reconstructions. Radiology 1995;194:871–7Google Scholar
85Martinez Del Pero, M, Sivasothy, P. Vasculitis of the upper and lower airway. Best Pract Res Clin Rheumatol 2009;23:403–17CrossRefGoogle ScholarPubMed
86Screaton, NJ, Sivasothy, P, Flower, CD, Lockwood, CM. Tracheal involvement in Wegener's granulomatosis: evaluation using spiral CT. Clin Radiol 1998;53:809–15Google Scholar