IVRAN综合征及其治疗进展

特发性视网膜血管炎、动脉瘤和视神经视网膜炎(IRVAN)综合征是一种稀有但是公认的临床疾病。该综合征主要临床表现为视网膜血管炎、分支动脉动脉瘤、视神经视网膜动脉瘤,患者视力在患病后急剧下降,目前对于该疾病的诊断和临床分期等问题许多临床医生尚不熟悉。IRVAN的治疗上尚无明确的统一的指南,多数文献为个案病例报告,患者预后视力多相差很大。目前该病主要的治疗方案为,全视网膜激光光凝、糖皮质激素口服或眼内注射治疗、抗血管内皮生长因子(VEGF)药物治疗,免疫抑制剂治疗等方法。如何在不同时期,不同病情的患者中选择合理的疗法成为临床医生面临的主要困难。本文总结了近20年国内外所有IRVAN综合征报道的相关文献,以帮助临床医生更好的诊断和治疗IRVAN综合征提供参考。

IVRAN syndrome and progress in its treatment

Idiopathic retinal vasculitis,aneurysms and neuroretinitis (IRVAN)syndrome is a rare but recognized clinical disease. Important clinical manifestations of IRVAN syndrome include retinal vasculitis,branch artery aneurysm, and optic nerve retinal aneurysm. Visual acuity may decline rapidly,and many clinicians are not familiar with the diagnosis and its staging. There is no definite guideline for the treatment of IRVAN,and visual acuity prognosis varied significantly. Current mainstay treatments are whole retinal photocoagulation along with oral or intraocular steroid,anti-vascular endotheli-al growth factor (VEGF)therapy,and immunosuppressive therapy. To choose an appropriate treatment plan at different stage of the disease is a challenging task. This paper summarizes the relevant literatures of IRVAN syndrome to provide in-formation that could help clinicians with better diagnosis and treatment of IRVAN syndrome.