Primary Occlusive Panvasculitis with Segmental Periarterial Plaques.

Abstract

To describe a new clinical entity of bilateral occlusive panvasculitis with segmental periarterial plaques and its clinical course in two patients. 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 non-perfusion were observed. Both had recurrent episodes of vascular occlusions, normal brain MRI and audiology tests. Complete work-up including serology for infections, inflammatory markers and antibody titers were unremarkable. They were started on corticosteroids and immunosuppressant and there were no further vascular occlusions. Both developed neovascularization adjacent to areas of capillary non-perfusion. 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 last visit, visual acuity was 20/30 in both eyes for the first patient, 20/20 in the right 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 the constellation of findings constitute a novel clinical entity, occlusive panvasculitis with segmental periarterial plaques.