Cogan’s syndrome: new therapeutic approaches in the biological era

ABSTRACT

Introduction: Cogan’s syndrome (CS) is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. Treatment of CS could be challenging, and the only evidence-based data comes from case reports or series.

Areas covered: There have recently been several reports of new treatment strategy involving the use of biological disease-modifying anti-rheumatic drugs such as TNFα inhibitors, anti-CD20 or anti-IL6 receptor antibodies, in cases resistant to first- or second-line drugs.

Expert commentary: Corticosteroids are the cornerstone of CS therapy at disease onset and during acute phases of the disease. Conventional immunosuppressive therapy, such as methotrexate, could be used in relapsing patients or as a glucocorticoid sparing agent, but efficacy is often modest. The anti-TNFα monoclonal antibody Infliximab appears to be the most frequently used, leading to an improvement in hearing loss in 89% of the cases and allow corticosteroid tapering in 86% of the patients. The appropriate timing of Infliximab treatment has yet to be thoroughly investigated, but it seems to be more effective when started at an early stage of the disease. Efficacy of others anti-TNFα agents is controversial. Rituximab and Tocilizumab are a safe option, but results on hearing loss have still to be confirmed on larger patients’ cohorts.

KEYWORDS:

• Anti-TNFα
• biological agents
• Cogan’s syndrome
• hearing loss
• infliximab
• rituximab
• tocilizumab
• treatment

Article highlights

• Cogan’s syndrome is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. Histopathological data suggest a vasculitic nature of the disease.

• Due to the autoimmune inflammatory process, glucocorticoids are the first choice in the treatment of Cogan’s syndrome.

• Conventional immunosuppressive therapy, such as methotrexate, could be used in relapsing patients or as a glucocorticoid sparing agent. However, the efficacy is often modest.

• The anti-TNFα monoclonal antibody Infliximab appears to be the most frequently used, leading to an improvement in hearing loss in 89% of the cases and allow corticosteroid tapering in 86% of the patients.

• The use of others anti-TNFα agents is controversial.

• Rituximab and Tocilizumab are a safe option, but results on hearing loss has still to be confirmed on larger patients’ cohorts.

This box summarizes key points contained in the article.

Acknowledgments

The authors thank Frances Coburn for the English revision of the paper.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer Disclosures

Peer reviewers on this manuscript have no relevant financial relationships or otherwise to disclose.