Skip to main content
Top
Published in: European Archives of Oto-Rhino-Laryngology 1/2013

01-01-2013 | Miscellaneous

Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener’s)

Authors: Marcos Martinez Del Pero, Niels Rasmussen, Afzal Chaudhry, Piyush Jani, David Jayne

Published in: European Archives of Oto-Rhino-Laryngology | Issue 1/2013

Login to get access

Abstract

The objective of this prospective cross-sectional study is to describe the clinical otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener’s) (GPA) in a prospective cohort. All patients suffering from GPA seen in a tertiary centre between March 2007 and November 2008 had a detailed clinical assessment by an ENT surgeon of their ear, nose and head and neck complaints. An evaluation of whether there was disease activity and/or infection in each ENT area was made using the European Vasculitis Study Group guidelines. The number of patients assessed was 144. The proportion of female patients was 47 % (n = 69) and the median age was 57.7 years (IQ range 42.5–68.5). The prevalence of ENT involvement was 87 % (125/144). Hearing loss and abnormal tympanic membrane appearance were more common in patients with active disease and no infection (7/8 and 6/8, respectively, in active disease cf. 59/131 and 52/131, respectively, in remission). Nasal crusting was the most common nasal complaint recorded (52/144, 36 %) and bloody rhinorrhoea was the most common symptom in patients with disease activity. Rhinoscopy was highly sensitive in diagnosing disease activity (100 %). Subglottic stenosis was the most common head and neck manifestation (27/121, 22 %) and 74 % were symptomatic. In conclusion, the pattern and frequency of clinical ENT manifestations in GPA have been described in a large patient cohort. The use of tools readily available in the ENT clinic was essential to assess these patients accurately. This dataset will form the basis of an objective scoring system to measure disease activity in the ENT system.
Literature
1.
go back to reference Falk RJ, Gross WL, Guillevin L, Hoffman G, Jayne DR, Jennette JC, Kallenberg CG, Luqmani R, Mahr AD, Matteson EL, Merkel PA, Specks U, Watts R (2011) Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Ann Rheum Dis 70(4):704. doi:10.1136/ard.2011.150714 PubMedCrossRef Falk RJ, Gross WL, Guillevin L, Hoffman G, Jayne DR, Jennette JC, Kallenberg CG, Luqmani R, Mahr AD, Matteson EL, Merkel PA, Specks U, Watts R (2011) Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Ann Rheum Dis 70(4):704. doi:10.​1136/​ard.​2011.​150714 PubMedCrossRef
2.
go back to reference Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116(6):488–498PubMed Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116(6):488–498PubMed
3.
go back to reference Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, Peel ET, Wood JB (1992) Wegener’s granuloma. 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 83(302):427–438PubMed Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, Peel ET, Wood JB (1992) Wegener’s granuloma. 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 83(302):427–438PubMed
5.
go back to reference Srouji IA, Andrews P, Edwards C, Lund VJ (2007) Patterns of presentation and diagnosis of patients with Wegener’s granulomatosis: ENT aspects. J Laryngol Otol 121(7):653–658PubMedCrossRef Srouji IA, Andrews P, Edwards C, Lund VJ (2007) Patterns of presentation and diagnosis of patients with Wegener’s granulomatosis: ENT aspects. J Laryngol Otol 121(7):653–658PubMedCrossRef
6.
go back to reference Lane SE, Watts RA, Shepstone L, Scott DG (2005) Primary systemic vasculitis: clinical features and mortality. Q J Med 98(2):97–111CrossRef Lane SE, Watts RA, Shepstone L, Scott DG (2005) Primary systemic vasculitis: clinical features and mortality. Q J Med 98(2):97–111CrossRef
7.
go back to reference Ahmad I, Lee WC, Nagendran V, Wilson F, Shortridge RT (2000) Localised Wegener’s granulomatosis in otolaryngology: a review of six cases. ORL; Journal for oto-rhino-laryngology and its related specialties 62(3):149–155PubMedCrossRef Ahmad I, Lee WC, Nagendran V, Wilson F, Shortridge RT (2000) Localised Wegener’s granulomatosis in otolaryngology: a review of six cases. ORL; Journal for oto-rhino-laryngology and its related specialties 62(3):149–155PubMedCrossRef
8.
go back to reference Bakheit AM, Behan PO (1990) Localised Wegener’s granulomatosis–a difficult and delayed diagnosis. Clinical report and literature review. Ir J Med Sci 159(2):55–57PubMedCrossRef Bakheit AM, Behan PO (1990) Localised Wegener’s granulomatosis–a difficult and delayed diagnosis. Clinical report and literature review. Ir J Med Sci 159(2):55–57PubMedCrossRef
9.
go back to reference Watts RA, Lane S, Scott DG (2005) What is known about the epidemiology of the vasculitides? Best Pract Res 19(2):191–207CrossRef Watts RA, Lane S, Scott DG (2005) What is known about the epidemiology of the vasculitides? Best Pract Res 19(2):191–207CrossRef
11.
go back to reference Holle JU, Gross WL, Latza U, Noelle B, Ambrosch P, Heller M, Fertmann R, Reinhold-Keller E (2010) Improved outcome of 445 Wegener’s granulomatosis patients in a German vasculitis center over four decades. Arthr Rheum. doi:10.1002/art.27763 Holle JU, Gross WL, Latza U, Noelle B, Ambrosch P, Heller M, Fertmann R, Reinhold-Keller E (2010) Improved outcome of 445 Wegener’s granulomatosis patients in a German vasculitis center over four decades. Arthr Rheum. doi:10.​1002/​art.​27763
12.
go back to reference Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, Peikert T, Hoffman GS, Merkel PA, Spiera R, Spiera R, Spiera R, St Clair EW, Davis JC Jr, McCune WJ, Tibbs AK, Ytterberg SR, Stone JH, Specks U (2007) ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med 120(7):643.e9–643.e14. doi:10.1016/j.amjmed.2006.08.016 CrossRef Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, Peikert T, Hoffman GS, Merkel PA, Spiera R, Spiera R, Spiera R, St Clair EW, Davis JC Jr, McCune WJ, Tibbs AK, Ytterberg SR, Stone JH, Specks U (2007) ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med 120(7):643.e9–643.e14. doi:10.​1016/​j.​amjmed.​2006.​08.​016 CrossRef
13.
go back to reference De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, Gross WL, Luqmani R, Jayne DR (2005) Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthr Rheum 52(8):2461–2469. doi:10.1002/art.21142 CrossRef De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, Gross WL, Luqmani R, Jayne DR (2005) Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthr Rheum 52(8):2461–2469. doi:10.​1002/​art.​21142 CrossRef
14.
go back to reference Stone JH (2003) Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthr Rheum 48(8):2299–2309. doi:10.1002/art.11075 CrossRef Stone JH (2003) Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthr Rheum 48(8):2299–2309. doi:10.​1002/​art.​11075 CrossRef
15.
go back to reference Rasmussen N, Petersen J, Jensen H, Andersen V (1990) Histopathological findings in biopsies from patients with Wegener’s granulomatosis. APMIS Suppl 19:15–16CrossRef Rasmussen N, Petersen J, Jensen H, Andersen V (1990) Histopathological findings in biopsies from patients with Wegener’s granulomatosis. APMIS Suppl 19:15–16CrossRef
16.
go back to reference Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D (2007) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66(2):222–227. doi:10.1136/ard.2006.054593 PubMedCrossRef Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D (2007) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66(2):222–227. doi:10.​1136/​ard.​2006.​054593 PubMedCrossRef
17.
go back to reference Jayne D (2004) The clinical features and pathology of vasculitis associated with anti-myeloperoxidase autoantibodies. Jpn J Infect Dis 57(5):S16–S17PubMed Jayne D (2004) The clinical features and pathology of vasculitis associated with anti-myeloperoxidase autoantibodies. Jpn J Infect Dis 57(5):S16–S17PubMed
18.
go back to reference Scadding GK, Durham S (2008) Allergic rhinitis. In: Gleeson MJ (ed) Scott-Brown’s Otorhinolaryngology: Head and neck surgery, vol 2. 7th edn. Hodder Arnold, London, UK, p 1393 Scadding GK, Durham S (2008) Allergic rhinitis. In: Gleeson MJ (ed) Scott-Brown’s Otorhinolaryngology: Head and neck surgery, vol 2. 7th edn. Hodder Arnold, London, UK, p 1393
19.
go back to reference Hellmich B, Flossmann O, Gross WL, Bacon P, Cohen-Tervaert JW, Guillevin L, Jayne D, Mahr A, Merkel PA, Raspe H, Scott DG, Witter J, Yazici H, Luqmani RA (2007) EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 66(5):605–617. doi:10.1136/ard.2006.062711 PubMedCrossRef Hellmich B, Flossmann O, Gross WL, Bacon P, Cohen-Tervaert JW, Guillevin L, Jayne D, Mahr A, Merkel PA, Raspe H, Scott DG, Witter J, Yazici H, Luqmani RA (2007) EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 66(5):605–617. doi:10.​1136/​ard.​2006.​062711 PubMedCrossRef
20.
go back to reference Flossmann O, Bacon P, de Groot K, Jayne D, Rasmussen N, Seo P, Westman K, Luqmani R (2007) Development of comprehensive disease assessment in systemic vasculitis. Ann Rheum Dis 66(3):283–292PubMed Flossmann O, Bacon P, de Groot K, Jayne D, Rasmussen N, Seo P, Westman K, Luqmani R (2007) Development of comprehensive disease assessment in systemic vasculitis. Ann Rheum Dis 66(3):283–292PubMed
21.
go back to reference Exley AR, Bacon PA, Luqmani RA, Kitas GD, Gordon C, Savage CO, Adu D (1997) Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 40(2):371–380PubMedCrossRef Exley AR, Bacon PA, Luqmani RA, Kitas GD, Gordon C, Savage CO, Adu D (1997) Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 40(2):371–380PubMedCrossRef
22.
go back to reference Jennings CR, Jones NS, Dugar J, Powell RJ, Lowe J (1998) Wegener’s granulomatosis–a review of diagnosis and treatment in 53 subjects. Rhinology 36(4):188–191PubMed Jennings CR, Jones NS, Dugar J, Powell RJ, Lowe J (1998) Wegener’s granulomatosis–a review of diagnosis and treatment in 53 subjects. Rhinology 36(4):188–191PubMed
23.
go back to reference Kornblut AD, Wolff SM, Fauci AS (1982) Ear disease in patients with Wegener’s granulomatosis. Laryngoscope 92(7 Pt 1):713–717PubMedCrossRef Kornblut AD, Wolff SM, Fauci AS (1982) Ear disease in patients with Wegener’s granulomatosis. Laryngoscope 92(7 Pt 1):713–717PubMedCrossRef
25.
26.
go back to reference Blanchet C, Pommie C, Mondain M, Berr C, Hillaire D, Puel JL (2008) Pure-tone threshold description of an elderly French screened population. Otol Neurotol 29(4):432–440. doi:10.1097/MAO PubMedCrossRef Blanchet C, Pommie C, Mondain M, Berr C, Hillaire D, Puel JL (2008) Pure-tone threshold description of an elderly French screened population. Otol Neurotol 29(4):432–440. doi:10.​1097/​MAO PubMedCrossRef
27.
go back to reference Sproson E, Jones N, Al-Deiri M, Lanyon P (2007) Lessons learnt in the management of Wegener’s Granulomatosis: long-term follow-up of 60 patients. Rhinology 45:63–67PubMed Sproson E, Jones N, Al-Deiri M, Lanyon P (2007) Lessons learnt in the management of Wegener’s Granulomatosis: long-term follow-up of 60 patients. Rhinology 45:63–67PubMed
28.
go back to reference Kornblut AD, Wolff SM, deFries HO, Fauci AS (1980) Wegener’s granulomatosis. Laryngoscope 90(9):1453–1465PubMedCrossRef Kornblut AD, Wolff SM, deFries HO, Fauci AS (1980) Wegener’s granulomatosis. Laryngoscope 90(9):1453–1465PubMedCrossRef
29.
go back to reference Stegeman CA, Tervaert JW, Sluiter WJ, Manson WL, de Jong PE, Kallenberg CG (1994) Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med 120(1):12–17PubMed Stegeman CA, Tervaert JW, Sluiter WJ, Manson WL, de Jong PE, Kallenberg CG (1994) Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med 120(1):12–17PubMed
30.
go back to reference Langford CA, Sneller MC, Hallahan CW, Hoffman GS, Kammerer WA, Talar-Williams C, Fauci AS, Lebovics RS (1996) Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Arthr Rheum 39(10):1754–1760CrossRef Langford CA, Sneller MC, Hallahan CW, Hoffman GS, Kammerer WA, Talar-Williams C, Fauci AS, Lebovics RS (1996) Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Arthr Rheum 39(10):1754–1760CrossRef
31.
go back to reference Abou-Elhmd KA, Hawthorne MR, Flood LM (1996) Cochlear implantation in a case of Wegener’s granulomatosis. J Laryngol Otol 110(10):958–961PubMedCrossRef Abou-Elhmd KA, Hawthorne MR, Flood LM (1996) Cochlear implantation in a case of Wegener’s granulomatosis. J Laryngol Otol 110(10):958–961PubMedCrossRef
32.
go back to reference Congdon D, Sherris DA, Specks U, McDonald T (2002) Long-term follow-up of repair of external nasal deformities in patients with Wegener’s granulomatosis. Laryngoscope 112(4):731–737PubMedCrossRef Congdon D, Sherris DA, Specks U, McDonald T (2002) Long-term follow-up of repair of external nasal deformities in patients with Wegener’s granulomatosis. Laryngoscope 112(4):731–737PubMedCrossRef
33.
go back to reference Pirsig W, Pentz S, Lenders H (1993) Repair of saddle nose deformity in Wegener’s granulomatosis and ectodermal dysplasia. Rhinology 31(2):69–72PubMed Pirsig W, Pentz S, Lenders H (1993) Repair of saddle nose deformity in Wegener’s granulomatosis and ectodermal dysplasia. Rhinology 31(2):69–72PubMed
34.
go back to reference Fechner FP, Faquin WC, Pilch BZ (2002) Wegener’s granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope 112(11):1945–1950PubMedCrossRef Fechner FP, Faquin WC, Pilch BZ (2002) Wegener’s granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope 112(11):1945–1950PubMedCrossRef
35.
go back to reference Hardwig PW, Bartley GB, Garrity JA (1992) Surgical management of nasolacrimal duct obstruction in patients with Wegener’s granulomatosis. Ophthalmology 99(1):133–139PubMed Hardwig PW, Bartley GB, Garrity JA (1992) Surgical management of nasolacrimal duct obstruction in patients with Wegener’s granulomatosis. Ophthalmology 99(1):133–139PubMed
36.
go back to reference DeRemee RA, McDonald TJ, Harrison EG Jr, Coles DT (1976) Wegener’s granulomatosis. Anatomic correlates, a proposed classification. Mayo Clin Proc 51(12):777–781PubMed DeRemee RA, McDonald TJ, Harrison EG Jr, Coles DT (1976) Wegener’s granulomatosis. Anatomic correlates, a proposed classification. Mayo Clin Proc 51(12):777–781PubMed
Metadata
Title
Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener’s)
Authors
Marcos Martinez Del Pero
Niels Rasmussen
Afzal Chaudhry
Piyush Jani
David Jayne
Publication date
01-01-2013
Publisher
Springer-Verlag
Published in
European Archives of Oto-Rhino-Laryngology / Issue 1/2013
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI
https://doi.org/10.1007/s00405-012-2110-8

Other articles of this Issue 1/2013

European Archives of Oto-Rhino-Laryngology 1/2013 Go to the issue