Dominik Kühn1, Charlotte Hospowsky1, Marcus Both2, Matthias Hey1, Martin Laudien3. 1. Department of Otorhinolaryngology, Head and Neck Surgery, University of Kiel, Germany. 2. Department of Radiology and Neuroradiology, University Hospital Schleswig-Holstein, Campus Kiel, Germany. 3. Department of Otorhinolaryngology, Head and Neck Surgery, University of Kiel, Germany. laudien@hno.uni-kiel.de.
Abstract
OBJECTIVES: Granulomatosis with polyangiitis is a rare autoimmune disease of the group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Involvement of the ear, nose and throat (ENT)-region is only described in a few case series and case reports. The objective of this study is to systematically characterise the ENT-involvement in a large series of GPA patients. METHODS: GPA patients examined in the Department of Otorhinolayngology of the Christian-Albrechts-University of Kiel between 1990 and 2012 were included. Diagnosis was based on histological, serological and clinical parameters. GPA patients were examined in a standardised way based on the Ear Nose and Throat Activity Score (ENTAS) or its precursor. Medical history, ENT examination, diagnostic findings (ear, nose) and cranial radiology were documented cumulatively. RESULTS: A total of 230 GPA patients were included in this study. Over 95% of them showed ENT-involvement. 59% of the patients showed nasal obstructions, 57% a loss of smell. A hearing loss was diagnosed in 23% of the patients, 50% involvement in MR or CT scans and 15% showed laryngeal involvement. CONCLUSIONS: The data of the largest monocentric study presented here demonstrate a frequent ENT-involvement in GPA patients. Rhinological and ontological manifestations are most common.
OBJECTIVES:Granulomatosis with polyangiitis is a rare autoimmune disease of the group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Involvement of the ear, nose and throat (ENT)-region is only described in a few case series and case reports. The objective of this study is to systematically characterise the ENT-involvement in a large series of GPA patients. METHODS: GPA patients examined in the Department of Otorhinolayngology of the Christian-Albrechts-University of Kiel between 1990 and 2012 were included. Diagnosis was based on histological, serological and clinical parameters. GPA patients were examined in a standardised way based on the Ear Nose and Throat Activity Score (ENTAS) or its precursor. Medical history, ENT examination, diagnostic findings (ear, nose) and cranial radiology were documented cumulatively. RESULTS: A total of 230 GPA patients were included in this study. Over 95% of them showed ENT-involvement. 59% of the patients showed nasal obstructions, 57% a loss of smell. A hearing loss was diagnosed in 23% of the patients, 50% involvement in MR or CT scans and 15% showed laryngeal involvement. CONCLUSIONS: The data of the largest monocentric study presented here demonstrate a frequent ENT-involvement in GPA patients. Rhinological and ontological manifestations are most common.
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