Otologic Manifestation in IgG4-Related Systemic Disease.
- Author:
Han Kyu CHO
1
;
Yun Jong LEE
;
Jin Haeng CHUNG
;
Ja Won KOO
Author Information
1. Department of Otorhinolaryngology, National Medical Center, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Immunoglobulin G;
Hearing loss;
Autoimmune disease
- MeSH:
Aged;
Autoimmune Diseases;
Female;
Hearing Disorders;
Hearing Loss;
Hearing Loss, Mixed Conductive-Sensorineural;
Humans;
Immunoglobulin G;
Immunoglobulins;
Lacrimal Apparatus;
Lung;
Lymphocytes;
Methotrexate;
Methylprednisolone;
Otitis Media with Effusion;
Pancreatitis;
Plasma Cells;
Prednisolone;
Recurrence
- From:Clinical and Experimental Otorhinolaryngology
2011;4(1):52-54
- CountryRepublic of Korea
- Language:English
-
Abstract:
IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration. We report a case of a 66-year-old woman with IgG4-related hearing disorder, presenting with fluctuating mixed hearing loss with middle ear effusion. The serum IgG4 level was elevated and pathological examination revealed dense infiltration of IgG4-positive lymphocyte in the renal parenchyma, lung tissue and lacrimal gland. With intravenous methylprednisolone at a dose of 60 mg daily, improvement of hearing loss were observed. No recurrence was observed for 6 months with mainternance of prednisolone and methotrexate.