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.