Granulomatosis With Polyangiitis, Primarily Presented by Otological Symptoms: A Case Report and Literature Review

Arwa Obaid; Savvas Kourtidis; Annett Pudszuhn

doi:10.1055/s-0042-1746863

Laryngo-Rhino-Otologie, Table of Contents

CC BY-NC-ND 4.0 · Laryngorhinootologie 2022; 101(S 02): S243-S244
DOI: 10.1055/s-0042-1746863

Poster

Otology / Neurootology / Audiology: Inner ear

Granulomatosis With Polyangiitis, Primarily Presented by Otological Symptoms: A Case Report and Literature Review

Arwa Obaid

¹Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Department of Otorhinolaryngology, Head and Neck SurgeryBerlin

,

Savvas Kourtidis

¹Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Department of Otorhinolaryngology, Head and Neck SurgeryBerlin

,

Annett Pudszuhn

¹Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Department of Otorhinolaryngology, Head and Neck SurgeryBerlin

› Author Affiliations

Abstract

Full Text

Introduction

Granulomatosis with polyangiitis (GPA) is a rare, multisystemic, granulomatous vasculitis which is highly associated with anti-neutrophil cytoplasmic antibodies (cANCA). It is characterized by necrotizing granulomas, usually affecting the small blood vessels of airways and kidneys, but other organs, including eyes, joints and skin can also be involved.

Case Report

We report a case of a 68-year-old female, who initially presented with various refractory otologic symptoms followed by delayed sinonasal and arthritic involvement. Autoantibody tests demonstrated a positive proteinase-3 (PR-3) cANCA titers at 126.0 IU/ mL (normal < 10.0) and nasal biopsy confirmed the diagnosis of GPA.

Conclusion

A primary, solitary otological manifestation of granulomatosis with polyangiitis is uncommon; therefore, it should be considered in the differential diagnosis when symptoms progress rapidly or do not improve despite appropriate management.