Granulomatosis with polyangiitis presenting as isolated ear involvement: a case series and literature review
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View abstract on PubMed
Summary
This summary is machine-generated.Granulomatosis with polyangiitis (GPA) rarely presents with isolated ear symptoms. Early immunosuppressive therapy improves middle ear function in patients with GPA and otologic involvement.
Area Of Science
- Otolaryngology
- Rheumatology
- Immunology
Background
- Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease.
- GPA can affect various organs, including the ears, but isolated ear involvement as an initial presentation is uncommon.
Purpose Of The Study
- To detail the clinical features and outcomes of GPA patients with initial isolated ear symptoms.
- To highlight the importance of recognizing GPA in patients with unexplained otologic issues.
Main Methods
- A retrospective review of GPA patients treated at the University of Brescia, Italy (2002-2023).
- Inclusion criteria focused on patients whose first presentation was exclusively otologic manifestations.
Main Results
- Six out of 610 patients (0.8%) with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) had initial isolated ear involvement, all diagnosed with GPA.
- Common symptoms included otitis media with effusion, hearing loss (sensorineural or mixed), and dizziness.
- Two patients later developed systemic symptoms, and all showed partial recovery of middle ear function with immunosuppressive therapy.
Conclusions
- Isolated ear involvement is a rare initial manifestation of AAV, often presenting as refractory otitis media with hearing loss.
- Avoidance of surgery is crucial in suspected AAV cases to prevent iatrogenic damage.
- Immunosuppressive therapy is effective in improving otologic symptoms in GPA.
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