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Otologic Manifestation in IgG4-Related Systemic Disease
Han-Kyu Cho1, Yun Jong Lee, Jin-Haeng Chung
1Department of Otorhinolaryngology, National Medical Center, Seoul, Korea.
Clinical and Experimental Otorhinolaryngology
|April 5, 2011
Summary
Immunoglobulin G4 (IgG4)-related hearing disorder is a multi-organ condition. This case study shows successful treatment of IgG4-related hearing loss with corticosteroids and methotrexate.
Area of Science:
- Immunology
- Otolaryngology
- Rheumatology
Background:
- Immunoglobulin G4 (IgG4)-related disease is a chronic, fibrosing condition affecting multiple organs.
- It is characterized by elevated serum IgG4 levels and IgG4-positive plasma cell infiltration in affected tissues.
- Hearing impairment can be a presenting symptom, often associated with middle ear effusion.
Purpose of the Study:
- To report a case of a 66-year-old woman diagnosed with IgG4-related hearing disorder.
- To describe the clinical presentation, diagnostic findings, and treatment outcomes.
- To highlight the effectiveness of immunosuppressive therapy in managing this condition.
Main Methods:
- Clinical case presentation of a 66-year-old female patient.
- Diagnostic evaluation included audiometry, imaging, and histopathological examination of affected tissues (renal parenchyma, lung, lacrimal gland).
- Treatment involved intravenous methylprednisolone followed by maintenance therapy with prednisolone and methotrexate.
Main Results:
- The patient presented with fluctuating mixed hearing loss and middle ear effusion.
- Elevated serum IgG4 levels and IgG4-positive lymphocyte infiltration were confirmed in multiple organs.
- Significant improvement in hearing loss was observed after initiating intravenous methylprednisolone therapy.
- No recurrence was noted during a 6-month follow-up period with maintenance therapy.
Conclusions:
- IgG4-related hearing disorder is a treatable condition that can significantly impact auditory function.
- Early diagnosis and prompt immunosuppressive treatment, including corticosteroids and steroid-sparing agents like methotrexate, are crucial for favorable outcomes.
- This case underscores the importance of considering IgG4-related disease in patients with unexplained hearing loss and multi-organ involvement.
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