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Recurrent Mastoiditis Mimics IgG4 Related Disease: A Potential Diagnostic Pitfall
Vikram Deshpande1, Nicolas A Zane2, Stefan Kraft2
1Department of Pathology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA, 02114, USA. vdeshpande@partners.org.
Head and Neck Pathology
|April 20, 2016
Summary
Immunoglobulin G4-related disease (IgG4-RD) can cause mastoiditis, presenting with varied symptoms. While distinct, IgG4-related mastoiditis is uncommon and requires careful diagnosis to rule out infection.
Area of Science:
- Otolaryngology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a fibrotic condition characterized by specific histological features and elevated serum IgG4.
- Mastoiditis, an inflammation of the mastoid bone, can have various causes.
Observation:
- This study reports three cases of IgG4-RD affecting the mastoid sinus, presenting with symptoms like tinnitus, hearing loss, and cranial nerve palsies.
- Histological examination revealed characteristic IgG4-RD features, and patients responded to immunosuppressive therapy.
Findings:
- A review of 162 mastoiditis cases identified two instances meeting IgG4-RD criteria, but these were ultimately attributed to severe infections.
- IgG4-related mastoiditis is confirmed as a rare but distinct entity.
Implications:
- The findings highlight IgG4-related mastoiditis as a potential, albeit uncommon, cause of recurrent mastoiditis.
- Accurate diagnosis necessitates excluding infectious etiologies, emphasizing the importance of differential diagnosis in mastoiditis cases.
