Evaluation of peripheral retinal vasculitis in retinitis pigmentosa using wide-field fluorescein angiography

Abstract

 

Purpose

 

Peripheral retinal vascular leakage in patients with retinitis pigmentosa (RP) may be more common than previously thought and may be indicative of retinal vasculitis and an inflammatory component to the pathogenesis of photoreceptor degeneration in RP. Cystoid macular edema, a common complication of RP, may be associated with this vasculitis.

 

Methods

 

We performed a retrospective analysis of RP patients that received Optos wide-field photography and wide-field fluorescein angiogrophy (FA) studies and macular optical coherence tomography (OCT) scans at UPMC Eye Center. Fifty eyes of 25 patients were included. All images were de-identified and read by two senior retina faculty members. Photographs and FA images were examined for hemorrhages, exudate, vascular sheathing, peripheral retinal leakage, capillary nonperfusion, disc leakage, and neovascularization. OCT scans were examined for the presence of cystoid macular edema.

 

Results

 

Peripheral vascular leakage was present in at least one eye of 15 out of the 25 patients we examined. Vascular sheathing was present in four patients and peripheral telangiectasias were observed in six patients. Retinal neovascularization (rather than NVE) was observed in one patient. Thirteen out of 25 patients had a history of CME. Of these 13 patients, eight were found to have peripheral vascular leakage.

 

Conclusions

 

Retinitis pigmentosa is a family of genetic syndromes characterized by the progressive degeneration and dysfunction of the rod and cone photoreceptors. The pathogenesis of this degeneration is not well understood, but recent studies have focused on the inflammatory response as a possible component. Using Optos wide-field photography and wide-field fluorescein angiography, we found peripheral vascular leakage, indicative of vasculitis, in 15 of 25 patients, supporting an inflammatory component to the pathogenesis of photoreceptor loss. Yoshida et al. recently reported vitreous cell in 37.3% of 509 RP patients they examined, as well as increased concentrations of pro-inflammatory cytokines and chemokines. Heckenlively et al. reported that 90% of 30 RP patients who presented with CME had circulating anti-retinal antibodies. Further characterization of retinal inflammation in RP may provide a target for future therapy.

 

 

Leaking peripheral vasculature in a patient with retinitis pigmentosa captured by Optos wide-field fluorescein angiography

 

Keywords: 696 retinal degenerations: hereditary • 702 retinitis • 552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound)