Uveitis Unit, Department of Ophthalmology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Autoimmun Rev. 2017 Mar;16(3):237-243. doi: 10.1016/j.autrev.2017.01.006. Epub 2017 Jan 27.
Isolated retinal vasculitis (IRV) is an inflammatory condition of unknown etiology confined to the retinal vessels. In contrast to secondary retinal vasculitis (RV), IRV has not been well characterized.
To describe and characterize isolated forms of RV.
We performed a retrospective review (2006-2016) of IRV patients from a multidisciplinary Uveitis Unit. RV diagnosis was based on funduscopic and fluorescein angiography findings. To distinguish between secondary RV and IRV, evaluations included clinical assessment, several inflammatory, autoimmune and microbiological laboratory markers, and a chest radiography. Ophthalmological features at disease onset, therapeutic interventions, ocular relapses, visual outcomes and laboratory findings were recorded. Our cases were subsequently compared with those from a literature review.
Among 192 patients with RV, 11 (5.7%) were diagnosed with IRV. Seven patients with initially presumed IRV were reclassified as secondary after further evaluation. IRV generally affected adult women. Bilateral ocular involvement and retinal phlebitis were common findings. 72% of patients presented with visual loss, which was severe in 27%. Treatments used included systemic glucocorticoids (82%), additional immunosuppressive agents (27%), intravitreal therapy (37%), panretinal photocoagulation (37%) and pars plana vitrectomy (26%). The annual relapse rate was 0.46. Although final visual acuity was considered good in 86%, 45% experienced worsening and only 27% improved.
IRV is a rare sight-threatening condition. Despite intensive local and systemic immunosuppressive treatment, visual improvement is observed in only 27% of cases. When IRV is suspected, a differential diagnosis excluding a systemic disease is always warranted. A multidisciplinary approach and a guided clinical, laboratory and imaging evaluation have proven to be useful to distinguish retinal single-organ vasculitis from secondary forms of RV.
孤立性视网膜血管炎（IRV）是一种病因不明的炎症性疾病，局限于视网膜血管。与继发性视网膜血管炎（RV）不同，IRV 尚未得到很好的描述。
描述和特征化孤立性 RV 形式。
我们对来自多学科葡萄膜炎单位的 IRV 患者进行了回顾性研究（2006-2016 年）。RV 的诊断基于眼底和荧光素血管造影的发现。为了区分继发性 RV 和 IRV，评估包括临床评估、几种炎症、自身免疫和微生物学实验室标志物，以及胸部 X 光检查。记录疾病发作时的眼科特征、治疗干预、眼部复发、视力结果和实验室发现。随后将我们的病例与文献综述中的病例进行比较。
在 192 例 RV 患者中，有 11 例（5.7%）被诊断为 IRV。7 例最初被认为是 IRV 的患者在进一步评估后被重新归类为继发性。IRV 通常影响成年女性。双眼受累和视网膜静脉炎是常见的发现。72%的患者出现视力丧失，其中 27%的患者视力严重丧失。使用的治疗方法包括全身糖皮质激素（82%）、额外的免疫抑制剂（27%）、玻璃体内治疗（37%）、全视网膜光凝（37%）和经睫状体平坦部玻璃体切除术（26%）。年复发率为 0.46。尽管 86%的患者最终视力被认为良好，但仍有 45%的患者视力恶化，只有 27%的患者视力改善。
IRV 是一种罕见的威胁视力的疾病。尽管进行了局部和全身免疫抑制治疗，但只有 27%的病例观察到视力改善。当怀疑 IRV 时，排除系统性疾病的鉴别诊断始终是必要的。多学科方法和有指导的临床、实验室和影像学评估已被证明有助于将单一器官视网膜血管炎与继发性 RV 区分开来。
