Chong Yu Jeat, Logeswaran Abison, Sarmad Ambreen, Damato Erika Marie
Birmingham Midland Eye Centre, 76 Dudley Rd., Birmingham, B18 7QH, UK.
Moorfields Eye Hospital, 162 City Road, London, EC1V 2PD, UK.
Graefes Arch Clin Exp Ophthalmol. 2019 May;257(5):1013-1018. doi: 10.1007/s00417-019-04291-5. Epub 2019 Mar 15.
The purpose of this study was to report the clinical presentation, disease progression, treatment and complications of IRVAN.
Case series PATIENTS: Six eyes from three patients were included.
All eyes were treated with pan-retinal photocoagulation (PRP). One eye received Ozurdex (dexamethasome implant) for persistent macular exudates and oedema. One eye received Avastin injections for retinal neovascularization. Oral steroids were given to all patients at some point during the disease process. One patient had additional immunosuppression with mycophenolate mofetil. Despite aggressive PRP, the visual outcomes varied widely. One patient maintained 6/6 vision bilaterally at 84 months follow-up. The second patient had progressive visual loss secondary to macular exudates and oedema, from 6/9 right eye, 6/6 left eye to 6/18 right eye, 6/60 left eye within 12 months despite Ozurdex injection. The third patient's vision at presentation was 6/5 right eye, and 6/4 left eye. Despite further interventions including Avastin and mycophenolate mofetil, he continued to have progressive neovascularization and recurrent vitreous haemorrhage. At 72 months, his vision had deteriorated to 6/60 right eye, 6/18 left eye.
The progression of IRVAN can vary greatly, in spite of aggressive treatment with PRP, oral and intravitreal steroids, immunosuppressant medication and anti-VEGF agents. The variation in disease progression occurs both within the same individual as well as between individuals. An individualised approach to therapy is advocated.
本研究旨在报告特发性视网膜血管炎、动脉瘤样扩张和视神经视网膜炎（IRVAN）的临床表现、疾病进展、治疗及并发症。
病例系列研究。患者：纳入3例患者的6只眼。
所有患眼均接受了全视网膜光凝（PRP）治疗。1只眼因黄斑持续性渗出和水肿接受了Ozurdex（地塞米松植入剂）治疗。1只眼因视网膜新生血管接受了阿瓦斯汀注射治疗。所有患者在病程中的某个阶段均接受了口服类固醇治疗。1例患者加用霉酚酸酯进行免疫抑制治疗。尽管进行了积极的PRP治疗，但视力预后差异很大。1例患者在84个月的随访中双眼视力维持在6/6。第2例患者因黄斑渗出和水肿导致视力逐渐下降，尽管注射了Ozurdex，但在12个月内右眼视力从6/9、左眼视力从6/6分别降至6/18和6/60。第3例患者初诊时右眼视力为6/5，左眼视力为6/4。尽管采取了包括阿瓦斯汀和霉酚酸酯在内的进一步干预措施，但他仍持续出现新生血管形成和反复玻璃体出血。在72个月时，他的视力已恶化为右眼6/60，左眼6/18。
尽管采用了PRP、口服和玻璃体内类固醇、免疫抑制药物及抗VEGF药物进行积极治疗，但IRVAN的病情进展仍可能有很大差异。疾病进展的差异既存在于同一个体内，也存在于个体之间。提倡个体化治疗方法。
