Purpose: To ascertain the clinical features and long-term visual prognosis of birdshot chorioretinopathy (BCR), and to identify patients at risk of visual loss.
Design: Retrospective noncomparative case series.
Participants: Fifty-five consecutive patients with HLA-A29-positive BCR who were identified in ophthalmology departments of the University Medical Center of Utrecht and The Eye Hospital Rotterdam, of whom 37 were observed for at least 5 years.
Intervention: A review of the medical and photographic and/or angiographic records of 55 patients with HLA-A29-positive BCR.
Main outcome measures: Numerous variables were compared, including age and gender distribution, onset and course of BCR, ocular manifestations, therapeutic strategies and their outcomes, complications, systemic diseases, visual acuity (VA), and features associated with poor visual outcome.
Results: Loss of VA was gradual; the number of affected eyes with VA less than 20/200 increased from 9 of 108 (8%) at onset to 22 of 73 (30%) at 5 years and 19 of 49 (39%) at 10 years of follow-up. The cause of compromised VA was predominantly macular edema and macular atrophy (42 of 55 [76% of cases]). We found strong associations between the VA at onset and visual outcome after 5 and 10 years (P = 0.005 and P = 0.006, respectively). Mean VA at the 5-year follow-up was significantly lower if macular leakage was observed on angiography (P<0.001). No differences in annual loss of VA were observed between patients treated by standard therapeutic modalities and untreated patients.
Conclusion: The visual prognosis of BCR in a spectrum of uveitis is poor, and the recommended therapeutic regimens have had no effect on long-term visual prognosis. New treatment strategies are needed for this blinding disorder.