European Archives of Oto-Rhino-Laryngology

, Volume 270, Issue 1, pp 345–354 | Cite as

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

  • Marcos Martinez Del PeroEmail author
  • Niels Rasmussen
  • Afzal Chaudhry
  • Piyush Jani
  • David Jayne


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.


Granulomatosis with polyangiitis Wegener’s granulomatosis ENT Assessment 



This work was supported by the Cambridge Biomedical Research Centre.


  1. 1.
    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 PubMedCrossRefGoogle Scholar
  2. 2.
    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):488–498PubMedGoogle Scholar
  3. 3.
    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):427–438PubMedGoogle Scholar
  4. 4.
    Reinhold-Keller E, Beuge N, Latza U, de Groot K, Rudert H, Nolle B, Heller M, Gross WL (2000) An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43(5):1021–1032. doi: 10.1002/1529-0131(200005)43:5<1021:AID-ANR10>3.0.CO;2-J PubMedCrossRefGoogle Scholar
  5. 5.
    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):653–658PubMedCrossRefGoogle Scholar
  6. 6.
    Lane SE, Watts RA, Shepstone L, Scott DG (2005) Primary systemic vasculitis: clinical features and mortality. Q J Med 98(2):97–111CrossRefGoogle Scholar
  7. 7.
    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):149–155PubMedCrossRefGoogle Scholar
  8. 8.
    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):55–57PubMedCrossRefGoogle Scholar
  9. 9.
    Watts RA, Lane S, Scott DG (2005) What is known about the epidemiology of the vasculitides? Best Pract Res 19(2):191–207CrossRefGoogle Scholar
  10. 10.
    Takala JH, Kautiainen H, Malmberg H, Leirisalo-Repo M (2008) Wegener’s granulomatosis in Finland in 1981–2000: clinical presentation and diagnostic delay. Scand J Rheumatol 37(6):435–438. doi: 10.1080/03009740802238366 PubMedCrossRefGoogle Scholar
  11. 11.
    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 Google Scholar
  12. 12.
    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 CrossRefGoogle Scholar
  13. 13.
    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 CrossRefGoogle Scholar
  14. 14.
    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 CrossRefGoogle Scholar
  15. 15.
    Rasmussen N, Petersen J, Jensen H, Andersen V (1990) Histopathological findings in biopsies from patients with Wegener’s granulomatosis. APMIS Suppl 19:15–16CrossRefGoogle Scholar
  16. 16.
    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 PubMedCrossRefGoogle Scholar
  17. 17.
    Jayne D (2004) The clinical features and pathology of vasculitis associated with anti-myeloperoxidase autoantibodies. Jpn J Infect Dis 57(5):S16–S17PubMedGoogle Scholar
  18. 18.
    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 1393Google Scholar
  19. 19.
    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 PubMedCrossRefGoogle Scholar
  20. 20.
    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):283–292PubMedGoogle Scholar
  21. 21.
    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):371–380PubMedCrossRefGoogle Scholar
  22. 22.
    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):188–191PubMedGoogle Scholar
  23. 23.
    Kornblut AD, Wolff SM, Fauci AS (1982) Ear disease in patients with Wegener’s granulomatosis. Laryngoscope 92(7 Pt 1):713–717PubMedCrossRefGoogle Scholar
  24. 24.
    Bakthavachalam S, Driver MS, Cox C, Spiegel JH, Grundfast KM, Merkel PA (2004) Hearing loss in Wegener’s granulomatosis. Otol Neurotol 25(5):833–837. doi: 00129492-200409000-00030 PubMedCrossRefGoogle Scholar
  25. 25.
    McDonald TJ, DeRemee RA (1983) Wegener’s granulomatosis. Laryngoscope 93(2):220–231PubMedCrossRefGoogle Scholar
  26. 26.
    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 PubMedCrossRefGoogle Scholar
  27. 27.
    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 45:63–67PubMedGoogle Scholar
  28. 28.
    Kornblut AD, Wolff SM, deFries HO, Fauci AS (1980) Wegener’s granulomatosis. Laryngoscope 90(9):1453–1465PubMedCrossRefGoogle Scholar
  29. 29.
    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):12–17PubMedGoogle Scholar
  30. 30.
    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):1754–1760CrossRefGoogle Scholar
  31. 31.
    Abou-Elhmd KA, Hawthorne MR, Flood LM (1996) Cochlear implantation in a case of Wegener’s granulomatosis. J Laryngol Otol 110(10):958–961PubMedCrossRefGoogle Scholar
  32. 32.
    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):731–737PubMedCrossRefGoogle Scholar
  33. 33.
    Pirsig W, Pentz S, Lenders H (1993) Repair of saddle nose deformity in Wegener’s granulomatosis and ectodermal dysplasia. Rhinology 31(2):69–72PubMedGoogle Scholar
  34. 34.
    Fechner FP, Faquin WC, Pilch BZ (2002) Wegener’s granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope 112(11):1945–1950PubMedCrossRefGoogle Scholar
  35. 35.
    Hardwig PW, Bartley GB, Garrity JA (1992) Surgical management of nasolacrimal duct obstruction in patients with Wegener’s granulomatosis. Ophthalmology 99(1):133–139PubMedGoogle Scholar
  36. 36.
    DeRemee RA, McDonald TJ, Harrison EG Jr, Coles DT (1976) Wegener’s granulomatosis. Anatomic correlates, a proposed classification. Mayo Clin Proc 51(12):777–781PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Marcos Martinez Del Pero
    • 1
    • 4
    Email author
  • Niels Rasmussen
    • 2
  • Afzal Chaudhry
    • 3
  • Piyush Jani
    • 1
  • David Jayne
    • 3
  1. 1.Department of Otolaryngology, Addenbrooke’s HospitalCambridge University Hospitals NHS Foundation TrustCambridgeUK
  2. 2.Department of OtolaryngologyRigshospitaletCopenhagenDenmark
  3. 3.Department of Medicine, Addenbrooke’s HospitalCambridge University Hospitals NHS Foundation TrustCambridgeUK
  4. 4.ENT Department, Box 48Addenbrooke’s HospitalCambridgeUK

Personalised recommendations