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Wegener's granulomatosis presenting as meningitis

  • G Thiel (a1), M Shakeel (a1) and K Ah-See (a1)

Abstract

Background:

Wegener's granulomatosis is a rare but well recognised autoimmune necrotising vasculitis. Presentation of disease in the head and neck is common and mostly consists of nasal crusting, blockage and bloody discharge. Neurological presentation is very uncommon.

Methods:

We report a patient who presented to the medical emergency services with signs and symptoms of meningitis, but who was eventually diagnosed with Wegener's granulomatosis. A literature search on this topic was carried out using Medline and Embase (1996 to 2011), searching for ‘Wegener's granulomatosis’ and ‘meningitis’.

Results:

After thorough neurological and medical investigation, a combination of brain computed tomography, lumbar puncture, nasal biopsy and laboratory results refuted the diagnosis of meningitis and confirmed the diagnosis of Wegener's granulomatosis.

Conclusion:

To the best of our knowledge, this is the first English-language case report of a patient with Wegener's granulomatosis presenting with symptoms of meningitis unconfirmed on computed tomography and lumbar puncture.

Copyright

Corresponding author

Address for correspondence: Dr Gundula Thiel, University Department of Otorhinolaryngology, Lauriston Building, Lauriston Place, Edinburgh EH3 9HA, Scotland, UK E-mail: gundulathiel@doctors.org.uk

References

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1Gottschlich, S, Ambrosch, P, Kramkowski, D, Laudien, M, Buchelt, T, Gross, WL et al. Head and neck manifestations of Wegener's granulomatosis. Rhinology 2006;44:227–33
2Lamprecht, P, Gross, WL. Wegener's granulomatosis. Herz 2004;29:4756
3Di Comite, G, Bozzolo, EP, Praderio, L, Tresoldi, M, Sabbadini, MG. Meningeal involvement in Wegener's granulomatosis is associated with localized disease. Clin Exp Rheumatol 2006;2 (suppl 41):S60–4
4Stone, 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. Arthritis Rheum 2003;48:2299–309
5Wegener, F. Of generalized septic vessel disease [in German]. Verh Dtsch Ges Pathol 1936;29:202–10
6Jayne, D. Update on the European Vasculitis Study Group trials. Curr Opin Rheumatol 2001;13:4855
7Reinhold-Keller, E, Herlyn, K, Wagner-Bastmeyer, R, Gross, WL. Stable incidence of primary systemic vasculitides over five years: results from the German vasculitis register. Arthritis Rheum 2005;53:93–9
8Reinhold-Keller, E, Zeidler, A, Gutfleisch, J, Peter, HH, Raspe, HH, Gross, WL. Giant cell arteritis is more prevalent in urban than in rural populations: results of an epidemiological study of primary systemic vasculitides in Germany. Rheumatology 2000;39:1396–402
9Devaney, 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;14:555–64
10Fauci, 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;98:7685
11Reinhold-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;43:1021–32
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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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