This protocol addresses Cogan's syndrome presenting with cochleo-vestibular involvement of moderate to severe severity — a setting where the choice and timing of treatment are critical to limiting irreversible damage.
Why this presentation requires urgent action: Unlike ocular involvement, cochleo-vestibular involvement in Cogan's syndrome carries a poor prognosis, and rapid introduction of immunosuppressive or biologic therapy is justified. In moderate to severe forms, treatment escalation beyond conventional approaches is indicated.
The protocol for this scenario centres on an anti-TNF biologic agent, used alone or alongside disease-modifying antirheumatic treatment.
DOI: 10.1016/j.revmed.2024.09.007
Unlike ocular involvement, cochleo-vestibular involvement has a poor prognosis and justifies the rapid introduction of immunosuppressive treatment or biologic therapy.
In moderate to severe forms or in case of failure or inadequate response, immunosuppressants are recommended (methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide) according to their use in vasculitis.
More recently, anti-TNFα agents, particularly infliximab, have been offered in addition to steroid therapy, either alone or in combination with disease-modifying antirheumatic drugs (DMARDs).
In the French series, the response of cochleo-vestibular involvement was significantly higher in patients treated with infliximab compared to those treated with conventional immunosuppressants and/or steroid therapy alone (80% vs. 39% and 35%, respectively).
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