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76th Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

04.05. - 08.05.2005, Erfurt

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Primary systemic vasculitides in ENT: a diagnostic challenge

Meeting Abstract

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  • corresponding author Lars-Uwe Scholtz - ENT-Univ.Clinic Würzburg, Würzburg
  • H Steffen - Eye-Univ.Clinic Würzburg, Würzburg
  • Konrad Schwager - ENT-Univ.Clinic Würzburg, Würzburg

Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie. 76. Jahresversammlung der Deutschen Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e.V.. Erfurt, 04.-08.05.2005. Düsseldorf, Köln: German Medical Science; 2005. Doc05hno339

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/hno2005/05hno229.shtml

Published: September 22, 2005

© 2005 Scholtz et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

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Background: Wegener’s granulomatosis is primary necrotizing ANCA associated systemic vasculitides with preferential involvement of small and medium-sized vessels.

In same case with isolated form the diagnosis of Wegener’s granulomatosis may be difficult with unreliable PR3- ANCA serology and ambiguous histological results.

Patients and methods: The files of all patients with is primary necrotizing ANCA associated systemic vasculitides (n=10) between 1998 and 2004 were evaluated with respect to clinical presentation, histological examinations and relevant laboratory data.

Results: PR3- ANCA positively is found in about 40% of the patients with localized Wegener’s granulomatosis. The histological pattern of organ involvement was positive in 33%. A localised form of Wegener’s granulomatosis had been present in 2 cases, the generalised in 8 cases. Most organ involvements occurred in the ear, nose, throat tract and kidney. Manifestinations occurred to a lesser extent in the skin and central nervous system (blindness of both eyes). Treatment followed the Fauci scheme.

Conclusion: The diagnosis of Wegener’s granulomatosis in isolated or generalised forms is most successful using the PR3-ANCA.