Elsevier

Autoimmunity Reviews

Volume 12, Issue 3, January 2013, Pages 396-400
Autoimmunity Reviews

Review
Cogan's syndrome: An autoimmune inner ear disease

https://doi.org/10.1016/j.autrev.2012.07.012Get rights and content
Under a Creative Commons license
open access

Abstract

Objectives

The objective of our study was to review our current knowledge of the aetiopathogenesis of Cogan's syndrome, including viral infection and autoimmunity, and to discuss disease pathogenesis with relevance to pharmacotherapy.

Systematic review methodology

Relevant publications on the aetiopathogenesis and pharmacotherapy of Cogan's syndrome from 1945 to 2012 were analysed.

Results and conclusions

Cogan's syndrome is a rare autoimmune vasculitis, and its pathogenesis is unknown. Infection, but primarily autoimmunity, may play contributing roles in the pathogenesis of this disease. It is characterised by ocular and audiovestibular symptoms similar to those of Meniere's syndrome. Approximately 70% of patients have systemic disease, of which vasculitis is considered the pathological mechanism. The immunologic theory is based on the release of auto-antibodies against corneal, inner ear and endothelial antigens, and of anti-nuclear cytoplasmic auto-antibodies (ANCA).

Corticosteroids are the first line of treatment, and multiple immunosuppressive drugs have been tried with varying degrees of success. Tumour necrosis factor (TNF)-alpha blockers are a category of immunosuppressive agents representing a recent novel therapeutic option in Cogan's syndrome.

Keywords

Cogan's syndrome
Autoimmune inner ear diseases
Vasculitis
Anti-neutrophil cytoplasmic auto-antibody
Autoimmunity
Aetiopathogenesis
Therapy

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