a1 describe an interesting case of Cogan's syndrome that improves following therapy with prednisone and methotrexate. Many features of this case, including the prolonged treatment course, the absence of vasculitis, and the steroid-sparing effect of methotrexate, resemble my earlier report. However, their patient appears to have responded to methotrexate more slowly than my patient, since 2 years and 3 months elapsed between the institution of the methotrexate treatment and the successful tapering of the prednisone, whereas in my report, a substantial improvement was noted after only 3 months of methotrexate treatment. It is possible that some variability exists in patient response to methotrexate.
The Pouchot et al case highlights the chronic course that Cogan's syndrome can follow. Others have reported that Cogan's syndrome can be extremely variable, lasting from several months to more than 15 years (1). Although some patients will respond to relatively short courses of prednisone (2), prolonged treatment is sometimes necessary. In these patients, steroid side effects are likely to become problematic. This report and my previous case report both suggest that treatment with methotrexate may be helpful in this setting.
As noted by Pouchot et al, Cogan's syndrome can be complicated by systemic vasculitis in 15-21% of patients (3,4). The vasculitis often occurs late, 3 weeks to 8 years after the onset of disease, with a mean of 7 months (4). Since the efficacy of methotrexate in Cogan's syndroms-associated vasculitis is unknown, we agree that all patients should be monitored expectantly. More aggressive therapy with drugs such as cyclosporine (5) may be indicated should vasculitis occur.
Finally, in both cases, arguments against natural disease fluctuations were presented. However, spontaneous disease remissions remain possible. I agree with Pouchot et a1 that additional experiences should be reported before widely recommending methotrexate. Further reports regarding the use of methotrexate or other steroid-sparing agents in Cogan's syndrome, or other forms of autoimmune hearing loss, would be welcome.