Studies have found that patients with birdshot chorioretinitis (BSCR) may need to be on oral corticosteroids for at least the first 2 years of treatment, despite the use of immunosuppressive therapies. Therefore, investigators evaluated the outcomes of BSCR treatment over the long term and determined rates of sustained drug-free remission as well as relapse.
This was a retrospective cohort study of 107 patients diagnosed with BSCR at 2 US-based tertiary academic centers between 2003 and 2017. Evaluated patient outcomes included corticosteroid-sparing success, ability to discontinue steroids, long-term drug-free remission, and relapse rates.
Successful steroid sparing was achieved in most patients, with 89.2% of patients discontinuing oral steroids by 48 months. However, based on the differences between the 2 centers studied, steroid sparing might be achievable as quickly as 6 months. Sustained drug-free remission was observed in 24.5% of patients at the 48-month follow-up visit; the relapse rate was 0.24/person-year, with a median time to relapse of 3.3 years. The most commonly used single agents were prednisone and mycophenolate mofetil; when 2 agents were used in combination, the most common second agent was a calcineurin inhibitor (e.g., cyclosporine, tacrolimus).
The limitations of the study are its retrospective design, the 4.6-year duration of follow-up (which limits the ability to evaluate sustained drug-free remission), and the lack of a standardized approach to treating BSCR. There is significant variability among physicians in monitoring disease activity, corticosteroid tapering schedules, and the choice of a second agent or the indications to start a second agent.
Results of this study indicate that with aggressive and early immunosuppressive medication management, patients with BSCR might be successfully weaned off corticosteroids as early as 6 months. This study is helpful in terms of prognosis for patients with a documented relapse rate and drug-free remission results.