Retinal Vasculitis Associated With Dermatomyositis Treated With Intravenous Steroids. (January 2022)

• Record Type: Journal Article
• Authors: Knapp, Austen N.; Arshad, Jawad I.; Martin, Daniel F.; Hajj-Ali, Rula; Baynes, Kimberly; Lowder, Careen Y.; Srivastava, Sunil K.
• Is Part Of: Journal of vitreoretinal diseases. Volume 6:Number 1(2022)
• Page Start: 75
• Page End: 79
• Publication Date: 2022-01
• Subjects: dermatomyositis -- inflammatory diseases -- intraretinal hemorrhages -- retinal vasculitis -- uveitis; Retina -- Diseases -- Periodicals; Vitreous body -- Diseases -- Periodicals; Retina -- Diseases; Vitreous body -- Diseases; Periodicals; 617.735005
• DOI: 10.1177/24741264211018960
• Languages: English
• ISSNs: 2474-1264

Abstract

Purpose: This work aims to present treatment and long-term follow-up of a 31-year-old woman with dermatomyositis who presented with hemorrhagic retinal vasculitis and macular edema. Methods: A retrospective case report is presented. Results: A 31-year-old woman with dermatomyositis treated with systemic immunosuppression was evaluated for acute, reduced vision. Best-corrected visual acuity was hand motion in the right eye and 20/200 in the left eye. Fundus examination revealed diffuse intraretinal hemorrhages, cotton-wool spots, and vascular sheathing with a frosted branch angiitis–like appearance. Optical coherence tomography revealed significant macular edema and subretinal fluid that quickly resolved after admission and treatment with intravenous steroids. Multimodal imaging at 7-year follow-up disclosed long-term sequelae including peripheral nonperfusion and retinal neovascularization. Conclusions: When vasculitis associated with dermatomyositis is treated aggressively with intravenous steroids at initial presentation, good visual acuity outcomes can be achieved, but long-term consequences of retinal nonperfusion and neovascularization persist.