Elsevier

American Journal of Ophthalmology

Volume 170, October 2016, Pages 190-196
American Journal of Ophthalmology

Original article
Birdshot Retinochoroidopathy: Prognostic Factors of Long-term Visual Outcome

https://doi.org/10.1016/j.ajo.2016.08.007Get rights and content

Purpose

To determine the prognostic factors of long-term visual outcome in birdshot retinochoroidopathy (BRC).

Methods

Design: Retrospective case series. Study Population: Successive HLA-A29+ BRC patients whose latest visit was between May and August 2013 at a single tertiary center (Pitié-Salpétrière Hospital, Paris). Observation Procedure: Endpoint visual status (remission or deterioration) was determined for each patient based on clinical and ancillary data from the latest visit including optical coherence tomography (OCT), automated visual field (AVF), and angiograms. Main Outcome Measure: Epidemiologic, clinical, OCT, AVF, angiographic, and electrophysiological data at baseline were correlated to final visual status.

Results

Fifty-five patients were included. Mean observation period was 8 years (range: 0.6-23 years). Mean disease duration was 9.8 years (range: 1.2-32.7 years). Female-to-male sex ratio was 1.6:1. Factors of good visual prognosis (remission vs deterioration) included at baseline: late age of disease onset (49.5 vs 45 years, P = .05), presence of vitreous inflammatory reactions >2+ (35.9% vs 6.2%, P = .04), vascular leakage on fluorescein angiograms (FA) (44.4% vs 12.5%, P = .03), absence of macular pigment epithelium atrophy on FA (88.9% vs 62.5%, P = .05), and presence of macular edema on OCT (33.3% vs 6.2%, P = .04). Preserved electrooculography light peak and Arden ratio (P = .06) and presence of choroidal spots on infracyanine green angiograms (80.0% vs 53.3%, P = .08) seemed associated with the best prognoses.

Conclusion

This study suggests a series of prognostic factors of long-term visual outcome in BRC. Keeping in mind the insidious evolution of the disease, knowledge of such prognostic factors should help tailor the treatment and monitoring of birdshot patients.

Section snippets

Methods

Institutional review board approvals for retrospective chart reviews were obtained commensurate with the respective institutional requirements before the beginning of the study. Described research was approved by the Ethics Committee of the French Society of Ophthalmology and adhered to the tenets of the Declaration of Helsinki. Fully informed consent was obtained for all patients. This hospital-based retrospective study reviewed the files of consecutive HLA-A29-positive BRC patients13 whose

Baseline Characteristics

Fifty-five white patients were included. Female-to-male sex ratio was 1.6:1 (62.0% female, 38.0% male). Mean age at diagnosis was 49.1 years (range: 30.3-73.9 years). At baseline, mean disease duration was 2.8 years (range: 0-24.4 years). Mean follow-up duration was 8 years (range: 0.6-22.9 years). At endpoint, mean disease duration was 9.8 years (range: 1.2-32.3 years).

Medical history consisted of high blood pressure in 27.0% of cases, hypercholesterolemia in 16.4% of cases, type 2 diabetes in

Discussion

Birdshot retinochoroidopathy is a rare form of posterior uveitis that is characterized by the variability of its phenotypes and unpredictability of its evolution, which makes standardization of patient care very challenging. In fact, no consensus regarding treatments (type or duration) or surveillance modalities has been defined to date.1 In addition, a study by Tomkins-Netzer and associates recently demonstrated that patients who received intermittent or short-term treatments showed more

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      Due to the progressive nature of BSCR, it is essential to have accurate methods of monitoring disease activity and measuring tissue damage. BSCR may in fact demonstrate a long insidious course (Touhami et al., 2016), and studies have demonstrated a progressive deterioration of both central and peripheral retinal function (Symes et al., 2015), even in the absence of clinically detectable inflammation (Jack et al., 2016). If undertreated, chronic inflammation may lead to retinal atrophy and a retinitis pigmentosa-like degeneration may even occur (Symes et al., 2015; Touhami et al., 2016).

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    Authors Sara Touhami and Christine Fardeau participated equally in this work.

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