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Updated: Aug 25, 2026
An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
[Wegener's granulomatosis (WG) presented with hearing loss and without positive serologic ANCA]
1Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde der Charité, Universitätsklinikum der Humboldt-Universität zu Berlin. oegoektas@hotmail.com
Background:
Wegener's granulomatosis (WG) is a granulomatous inflammation involving the upper and lower respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. In contrast to a generalised WG with glomerulonephritis initial or isolated forms of the upper respiratory tract may be a diagnostic challenge.
Patient:
We report the case of a 33 year old man with clinical signs of a limited WG exhibiting an imminent irreversible hearing loss, negative PR3-ANCA (anti neutrophil cytoplasmic antibodies with proteinase 3 as target) in serum and an ambiguous histology.
Conclusion:
In case of a chronic otitis media and rhinitis as well as signs of a labyrinthine deafness a limited form of a WG has to be taken into account, even with an ambiguous histology and negative PR3-ANCA. This diagnosis is supported by high inflammation parameters, e. g. ESR and CRP, exclusion of infectious cause and response to corticosteroids. A quick therapeutic intervention with corticosteroids and cyclophosphamide is required in order to interrupt the vasculitis of the inner ear with consequential deafness.
