Clin Exp Otorhinolaryngol.  2011 Mar;4(1):52-54.

Otologic Manifestation in IgG4-Related Systemic Disease

• Cho HK
• Lee YJ
• Chung JH
• Koo JW

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.

Keyword

Immunoglobulin G; Hearing loss; Autoimmune disease

MeSH Terms

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
Immunoglobulin G
Immunoglobulins
Methotrexate
Methylprednisolone
Prednisolone

Figure

• 

  Fig. 1 Change of hearing according to the immunosuppressive treatment. Black bars at the top indicate the period of immunosuppressive therapy.

• 

  Fig. 2 Immunoglobulin G4 (IgG4) immunoreactivity in lacrimal gland. Note strong immunoreactivity for IgG4 in the cytoplasm of infiltrated lymphocytes and plasma cells (×400).

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