PRIMARY OCCLUSIVE PANVASCULITIS WITH SEGMENTAL PERIARTERIAL PLAQUES

The purpose of this study was to describe a new clinical entity of bilateral occlusive panvasculitis with segmental periarterial plaques and its clinical course in two patients.

This was a retrospective chart review.

Two patients with no medical history of any systemic inflammatory diseases presented with bilateral segmental periarterial plaques (Kyrieleis plaques), cotton wool spots, and microaneurysms. Segmental leakage, staining, and vascular occlusions involved the arterioles, venules, and capillaries. Leakage from the superficial capillary plexus in some areas bordering deep capillary plexus nonperfusion was observed. Both had recurrent episodes of vascular occlusions, normal brain MRI, and audiology tests. Complete workup including serology for infections, inflammatory markers, and antibody titers was unremarkable. They were started on with corticosteroids and immunosuppressant, and there were no further vascular occlusions. Both developed neovascularization adjacent to the areas of capillary nonperfusion. The second patient also developed vitreomacular traction and cystoid macular edema. He required intravitreal anti-VEGF injection, sector laser photocoagulation, and underwent a vitrectomy with membrane peeling. At the last visit, the visual acuity was 20/30 in both eyes for the first patient and 20/20 in the right eye and 20/40 in the left eye for the second patient, 12 and 6.5 years after initial presentation, respectively.

Both patients presented with findings of bilateral panvasculitis with prominent segmental periarterial plaques and had repeated episodes of vascular occlusions before corticosteroid and immunosuppressants treatment, after which no additional occlusions were observed. We propose that the constellation of findings constitutes a novel clinical entity, occlusive panvasculitis with segmental periarterial plaques.