Otologic Manifestations and Progression in Patients with Wegener’s granulomatosis: A Survey in 55 Patients

Document Type : Original

Authors

1 Chronic Respiratory Diseases Research Center, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

2 Orthopedic Surgeon, Akhtar Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Abstract

Introduction:
Granulomatosis with polyangiitis (GPA; also known as Wegener’s granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otology manifestations in the disease course.
 
Materials and Methods:
In the current prospective study, patients with a diagnosis of GPA from 2012–2016 were included. 
A definitive diagnosis was made based on the history, physical examination (otomicroscopy, Rinne and Weber test), audiometry, tympanometry, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibody (C-ANCA and P-ANCA) investigations, and pathologic studies.
 
Results:
Twenty-seven male and 28 female patients aged 41.6±15.3 years were enrolled. Ear involvement was found in 20 patients (36.3%), and the most prevalent symptom was loss of hearing followed by otalgia and tinnitus. Tinnitus improved in none of the patients. The most prevalent sign was otitis serous followed by mastoiditis and external otitis. The most important audiometry finding was sensorineural hearing loss. Pathological studies using pulmonary samples were more useful for diagnosis.
 
Conclusions:
Precise clinical examination is crucial for the early diagnosis of GPA. Otological manifestations are common, especially loss of hearing and otitis serous, and can be the first sign of this disease. Early diagnosis can lead to better treatment of Wegener’s granulomatosis.

Keywords

Main Subjects

References

1. Falk RJ, Gross WL, Guillevin L, Hoffman G, Jayne DR, Jennette JC, et al. Granulomatosis with polyangitis (Wegener’s): an alternative name for Wegener’s Granulomatosis. Ann Rheum Dis 2011; 70(4):704.
2. Hoffman 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(6):488–98.
3. Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, et al. 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 1992; 83(302):427–38.
4. Reinhold-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(5):1021–1032.
5. Srouji IA, Andrews P, Edwards C, Lund VJ. Patterns of presentation and diagnosis of patients with Wegener’s Granulomatosis: ENT aspects. J Laryng Olotol 2007; 121(7):653–8.
6. Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: clinical features and mortality. Q J Med 2005; 98(2):97–111.
7. Lund V, Cambridge G. Immunological aspects of Wegener's Granulomatosis. In: Passali D, Veldman JE, Lim DJ, editors. New frontiers in immunobiology. The Netherlands: Kugler Publications; 2000. p. 195–207.
8. Ahmad I, Lee WC, Nagendran V, Wilson F, Shortridge RT. Localized Wegener’s Granulomatosis in otolaryngology: a review of six cases. ORL J Otorhinolaryngol Relat Spec. 2000; 62(3):149–55.
9. Bakheit AM, Behan PO. Localized Wegener’s Granulomatosis- a difficult and delayed diagnosis. Clinical report and literature review. Ir J Med Sci 1990; 159(2):55–7.
10. Takala 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(6): 435–8.
11. Holle JU, Gross WL, Latza U, Nölle B, Ambrosch P, Heller M, et al. Improved outcome of 445 Wegener’s Granulomatosis patients in a German vasculitis center over four decades. Arthritis Rheum. 2011; 63(1):257–66.
12. Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, et al. ANCA are detectable in nearly all patients with active severe Wegener’s Granulomatosis. Am J Med 2007; 120(7): 643.e9–14.
13. De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthr Rheum 2005; 52(8):2461–9.
14. Stone JH, Wegener's Granulomatosis Etanercept Trial Research Group. Limited versus severe Wegener’s Granulomatosis: baseline data on patients in the Wegener’s Granulomatosis etanercept trial.Arthr Rheum 2003;48(8):2299-309.
15. Rasmussen N, Petersen J, Jensen H, Andersen V. Histopathological findings in biopsies from patients with Wegener’s Granulomatosis. APMIS 1990; Suppl 19:15–16.
16. Watts 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 vasculitis’s and polyarteritisnodosa for epidemiological studies. Ann Rheum Dis 2007; 66(2):222–7.
17. Martinez Del Pero M, Rasmussen N, Chaudhry A, Jani P, Jayne D. Structured clinical assessment of the ear, nose and throat in patients with Granulomatosis with polyangitis (Wegener's). Eur Arch Otorhinolaryngol. 2013; 270(1):345–54.
18. Vega Braga FL, Machado de Carvalho G, Caixeta Guimarães A, Scaramussa L, Jordão Gusmão R. Otolaryngological manifestations of Wegener's disease. Acta Otorrinolaringol Esp 2013; 64(1):45–9.
19. Rodrigues CE, Callado MR, Nobre CA, Moura FE, Vieira RM, de Albuquerque LA, et al. Wegener's Granulomatosis: prevalence of the initial clinical manifestations--report of six cases and review of the literature. Rev Bras Reumatol 2010; 50(2):150–64.
Iranian Journal of Otorhinolaryngology
Volume 29, Issue 6 - Serial Number 95
November and December 2017
Pages 327-331

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