Abstract
Purpose :
Birdshot chorioretinopathy (BSCR) is a posterior uveitis with ovoid yellow-white choroidal lesions. If untreated, patients’ vision declines, so early diagnosis is critical. While indocyanine green angiography (ICGA) is used to identify subtle lesions, ICGA is not always available. Fluorescein angiography (FA) is more common and a contiguous, perinerve retinal vascular leakage pattern has been described in BSCR patients. The purpose of this study is to determine the sensitivity and positive predictive value (PPV) of a contiguous, perinerve retinal vascular leakage FA pattern for BSCR diagnosis.
Methods :
Mass General Brigham patients with a FA Common Procedural Terminology code and mention of BSCR in the free text of notes were identified. BSCR diagnosis required HLA-A29 positivity and typical lesions on fundus photos and/or ICGA hypofluorescent spots. Chart review was performed to confirm BSCR vs. other uveitis/retinal vasculitis diagnosis and symptom duration. The first FA was analyzed for a contiguous, perinerve pattern of leakage by two readers. A perinerve leakage pattern was defined as leakage primarily around the optic nerve and along the larger arcade vessels (Fig 1). We compared the rates of this FA pattern in BSCR vs. other posterior uveitis/retinal vasculitis patients using the chi-square test and determined the sensitivity and PPV of this FA pattern for BSCR diagnosis. All statistical analyses were performed in STATA.
Results :
54 BSCR patients and 42 patients with other posterior uveitis and/or retinal vasculitis were identified (Table 1). A perinerve FA pattern was more common in BSCR patients vs. patients without BSCR (53.7% vs. 4.76%, p=3.63 X 10-7). The sensitivity and specificity of the FA pattern were 53.7% and 95.2%, respectively. The PPV was 93.5%. BSCR patients with an FA perinerve pattern had a shorter time from symptom onset to FA vs. BSCR patients without the pattern (217 vs. 1510 days, p=.0015, t-test).
Conclusions :
An FA contiguous, perinerve retinal vascular leakage pattern is a useful tool to identify potential BSCR for further imaging and serological testing. This pattern is more common when the interval between symptom onset and FA is shorter.
This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.