How is Cogans syndrome treated?
Keratitis almost always responds to topical corticosteroid therapy. Topical cyclosporine is sometimes required, but the need for systemic steroids is rare (unless other ocular manifestations are present and dictate therapy). Note that keratitis resolves quickly on systemic corticosteroids. Therefore, an immediate eye exam is needed to document keratitis if the patient has other manifestation requiring high-dose corticosteroids. Otherwise, keratitis will be missed.
Vestibuloauditory dysfunction is less responsive to therapy in general. Oral prednisone is required and should be started at a dose of 1 mg/kg per day immediately. The usual tapering schedule is by 5 to 10 mg every 2 to 4 weeks, with a total duration of 4 to 6 months. Audiovestibular testing should be repeated 2 to 4 weeks after the initiation of treatment, and prednisone should be tapered quickly if no response is noted. A response to additional immunosuppression is unlikely if prednisone has failed. If an initial response is noted but relapse occurs as prednisone is tapered, a steroid-sparing agent should be initiated. Cochlear implantation can be considered for end-stage hearing loss.
Systemic manifestations such as vasculitis should be treated by standard immunosuppressants (e.g., cytotoxic or biologic agents, azathioprine, methotrexate, and mycophenolate mofetil).
