To enhance recognition of atypical presentations of ANCA-associated vasculitides and improve the diagnosis of Granulomatosis with Polyangiitis (GPA).
Approach:
Case Presentation: A 69-year-old female with a 3-month history of progressive bilateral hearing loss and otorrhea was initially diagnosed with acute mastoiditis but showed no response to treatment. Serological testing revealed elevated PR3-ANCA levels, leading to a diagnosis of …
Key Findings:
Isolated destructive mastoiditis as the initial presentation of GPA is exceedingly rare.
The patient did not respond to conventional antibiotics or surgical intervention.
Post-treatment with methylprednisolone and rituximab, the patient's symptoms improved, and PR3-ANCA titers decreased.
Interpretation:
In cases of refractory mastoiditis unresponsive to standard treatments, clinicians should consider underlying systemic vasculitides and perform early ANCA testing.
Limitations:
The case is based on a single patient, limiting generalizability.
Lack of long-term follow-up data on the patient's condition post-treatment.
Conclusion:
This case highlights the importance of early recognition and testing for GPA in patients with atypical presentations to prevent diagnostic delays and irreversible organ damage.
