Knapp Austen N, Arshad Jawad I, Martin Daniel F, Hajj-Ali Rula, Baynes Kimberly, Lowder Careen Y, Srivastava Sunil K
Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA.
Rheumatology Department, Cleveland Clinic, Cleveland, OH, USA.
J Vitreoretin Dis. 2021 Jun 15;6(1):75-79. doi: 10.1177/24741264211018960. eCollection 2022 Jan-Feb.
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.
A retrospective case report is presented.
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.
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.
本研究旨在介绍一名31岁皮肌炎女性患者的治疗及长期随访情况，该患者伴有出血性视网膜血管炎和黄斑水肿。
本文呈现了一例回顾性病例报告。
一名接受全身免疫抑制治疗的31岁皮肌炎女性因急性视力下降接受评估。最佳矫正视力右眼为手动，左眼为20/200。眼底检查发现视网膜弥漫性出血、棉絮斑和血管鞘，呈霜样分支血管炎样外观。光学相干断层扫描显示黄斑显著水肿和视网膜下液，入院并接受静脉注射类固醇治疗后迅速消退。7年随访的多模态成像显示存在长期后遗症，包括周边无灌注和视网膜新生血管形成。
当皮肌炎相关血管炎在初次就诊时积极采用静脉注射类固醇治疗时，可取得良好的视力结果，但视网膜无灌注和新生血管形成的长期后果依然存在。
