Vascular abnormalities in uveitis

Inflammation can involve several ocular structures, including the sclera, retina, and uvea, and cause vascular changes in these tissues. Although retinal vasculitis is the most common finding associated with uveitis involving the posterior segment, other vascular abnormalities may be seen in the retina. These include capillary nonperfusion and ischemia, vascular occlusions, preretinal neovascularization, microaneurysms and macroaneurysms, and telangiectasia. Moreover, vasoproliferative tumors and subsequent coat-like response can develop secondary to uveitis. Fluorescein angiography is ideal for the investigation of retinal vascular leakage and neovascularization, while optical coherence tomography angiography can provide depth resolved images from the superficial and deep capillary plexus and can demonstrate vascular remodeling. Choroidal vascular abnormalities primarily develop in the choriocapillaris or in the choroidal stroma and can appear as flow void in optical coherence tomography angiography and filling defect and vascular leakage in indocyanine green angiography. Extensive choriocapillaris nonperfusion in the presence of choroidal inflammation can increase the risk of choroidal neovascular membrane development. Iris vascular changes may manifest as dilation of vessels in stroma due to inflammation or rubeosis that is usually from ischemia in retinal periphery secondary to chronic inflammation. More severe forms of scleral inflammation, such as necrotizing scleritis, are associated with vascular occlusion in the deep episcleral plexus, which can lead to necrosis of sclera layer and uveal exposure.     

Use of Staurenghi lens angiography in the management of posterior uveitis

Purpose: Retinal vasculitis is an important component of posterior uveitis. It may be difficult to detect either clinically or with conventional angiography. We assessed the role of Staurenghi lens angiography (SLA) in the diagnosis and management of patients with posterior uveitis. Methods: A total of 26 patients attending the St Paul’s Eye Unit uveitis clinic with posterior uveitis underwent wide‐angle (150 degree) retinal angiography with the Heidelberg Retina Angiograph 2 Scanning Laser Ophthalmoscope using the Staurenghi SLO 230 contact lens. We determined the percentage of patients in whom this imaging modality assisted in diagnosing and quantifying the extent of vasculitis, planning treatment or monitoring disease activity. Results: Staurenghi lens angiography assisted in diagnosing and quantifying the extent of vasculitis in 62% of patients, and in planning laser photocoagulation or immunosuppression titration in 62% of patients and enhanced disease monitoring in 35% of patients. In 31% of cases, SLA revealed vasculitis that was not evident clinically. Conclusion: Staurenghi lens angiography is a valuable tool in the management of patients with posterior uveitis and can be used for the diagnosis, treatment and follow‐up of these patients.     

特发性视网膜血管炎、动脉瘤、视神经、视网膜炎综合征临床观察

目的 观察特发性视网膜血管炎、动脉瘤、视神经视网膜炎综合征（IRVAN综合征）的临床特征。 方法 回顾分析3例经全身系统检查、眼底彩色照相以及荧光素眼底血管造影（FFA）等检查确诊的IRVAN综合征患者的临床资料。 结果 3例患者均有特发性视网膜血管炎，而且是视网膜动脉的炎症，视盘及视网膜血管多发性大动脉瘤以及因视盘水肿、视盘周围渗出引起的视神经视网膜炎，2例患者周边部视网膜血管有闭塞区。 结论 IRVAN综合征的临床特征有特发性视网膜血管炎，视网膜和视盘动脉血管管壁上多发性大动脉瘤，以及由于血管炎症和动脉瘤引起的视网膜、视盘渗出、水肿以及由此产生的视神经视网膜炎。(中华眼底病杂志，2007，23：180-183)     

Retinal Vasculitis

Retinal vasculitis is a sight-threatening intraocular inflammation affecting the retinal vessels. It may occur as an isolated ocular condition, as a manifestation of infectious or neoplastic disorders, or in association with a systemic inflammatory disease. The search for an underlying etiology should be approached in a multidisciplinary fashion based on a thorough history, review of systems, physical examination, and laboratory evaluation. Discrimination between infectious and noninfectious etiologies of retinal vasculitis is important because their treatment is different. This review is based on recently published articles on retinal vasculitis and deals with its clinical diagnosis, its link with systemic diseases, and its laboratory investigation.     

Review for Diagnostics of the Year: Multimodal Imaging in Behçet Uveitis

Behçet disease is a chronic relapsing multisystem inflammatory disorder. Ocular involvement is characterized by a bilateral recurrent non-granulomatous panuveitis and retinal vasculitis. Posterior segment findings vary during the course of the disease, in parallel with the relapsing and remitting intraocular inflammation. Structural alterations occur with increased disease duration. Fluorescein angiography is the gold standard in revealing the extent and severity as well as the leaky and/or occlusive nature of retinal vasculitis. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualizing diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in patients with Behçet uveitis.     

视网膜血管炎

视网膜血管炎是一复杂眼底病,眼底表现为血管外白鞘,常伴玻璃体炎或视网膜脉络膜炎、病因除视网膜局部血管炎症外可伴有全身血管炎,甚至恶性肿瘤,因而要求眼科医师具有更广泛的知识,从眼部病变进而分析出全身疾病,及时进行治疗及转诊,不仅挽救视力,还可能拯救生命。本文就我国常见的感染性、非感染性、眼部特发性血管炎的诊治做一简要描述,并对可能伴有全身血管炎的病例就如何从病史、体格检查、实验室及影像学检查着手,逐步深入分析,找出诊治方向。     

特发性视网膜血管炎、动脉瘤、视神经视网膜炎综合征和Eales病荧光素眼底血管造影对比分析

目的 观察特发性视网膜血管炎、动脉瘤、视神经视网膜（IRVAN）综合征和Eales病荧光素眼底荧光血管造影（FFA）特征的异同。方法 回顾分析IRVAN综合征患者4例8只眼和Eales病患者43例68只眼的FFA检查资料。所有患者均行常规眼科检查,包括视力、眼压、裂隙灯显微镜、间接检眼镜眼底检查。4例Eales病患者单眼玻璃体积血眼底窥不清,行对侧眼检查,其他所有患者双眼常规彩色眼底照相、共焦激光扫描FFA检查。IRVAN综合征4例8只眼,均为双眼发病。男性1例, 女性3例;年龄16～43岁,平均年龄（27.00±12.93）岁。Eales病43例68只眼, 男性32例, 女性11例;年龄6～59岁,平均年龄（30.79±11.46）岁。双眼29例54只眼,单眼14例14只眼。两组患者眼底均可见视网膜血管白鞘或白线状改变、视网膜出血渗出、玻璃体积血。结果 FFA检查结果显示,IRVAN综合征8只眼后极部动、静脉均受累;均可见多发性视网膜大动脉瘤。周边部毛细血管闭塞形成无灌注区7只眼,占87.50%;视盘水肿荧光渗漏5只眼,占62.50%;视神经萎缩2只眼,25.00%;出血遮挡1只眼,占12.50%;视盘新生血管2只眼,占25.00%;视网膜新生血管4只眼,占50.00%;黄斑水肿4只眼,50.00%。Eales病43例68只眼中,所有患眼周边病变区视网膜静脉管壁渗漏;累及后极部静脉32只眼,占47.06%;动脉同时受累5只眼,占7.35%;周边部毛细血管闭塞形成无灌注区38只眼,占55.88%;视盘渗漏29只眼,占42.65%;视盘新生血管4只眼,占5.88%;视网膜新生血管26只眼,占38.24%;黄斑水肿15只眼,占22.06%。IRVAN综合征与Eales病患者在后极部动脉受累眼数、静脉受累眼数、动脉瘤眼数之间比较,差异有统计学意义(P均＜0.05);视盘渗漏、无灌注区、视盘和视网膜新生血管、黄斑水肿眼数之间比较,差异无统计学意义(χ2=0.479,1.449,0.068,1.676;P＞0.05)。结论 IRVAN综合征和Eales病均可发生视网膜动脉和静脉不同程度的炎性改变,并均可导致视盘水肿渗出。IRVAN综合征后极部视网膜动、静脉受累明显高于Eales病,特别是视盘旁及后极部特征性大动脉瘤样改变有助于IRVAN综合征的诊断及IRVAN综合征和Eales病的鉴别诊断。     

Retinal vasculitis

Inflammation involving the retinal vasculature may occur as an isolated finding unrelated to an identifiable underlying diagnosis, termed primary retinal vasculitis, or may be associated with a myriad of both ocular and systemic pathology. Retinal vasculitis is most accurately defined as vascular leakage and staining of vessel walls on fluorescein angiography, with or without the clinical appearance of fluffy, white perivascular infiltrates in the eye with evidence of inflammatory cells in the vitreous body or aqueous humor. Management of retinal vasculitis requires a dualistic approach: one, to control ocular inflammation and reverse a potentially sight-threatening condition, and two, to spare no effort in identifying and treating concomitant, and in many cases, life- threatening systemic disease.     

Imaging in retinal vascular disease: A review

Retinal vascular diseases represent a broad field of ocular pathologies. Retinal imaging is an important tool for diagnosis, prognosis and follow up of retinal vascular diseases. It includes a wide variety of imaging techniques ranging from colour fundus photography and optical coherence tomography to dynamic diagnostic options such as fluorescein angiography, and optical coherence tomography angiography. The newest developments in respective imaging techniques include widefield imaging to assess the retinal periphery, which is of especial interest in retinal vascular diseases. Automatic image analysis and artificial intelligence may support the image analysis and may prove valuable for prognostic purposes. This review provides a broad overview of the imaging techniques that have been used in the past, today and maybe in the future to stage and monitor retinal vascular disease with focus on the main disease entities including diabetic retinopathy, retinal vein occlusion, and retinal artery occlusion.     

Retinal vasculitis: A framework and proposal for a classification system

Retinal vasculitis, a poorly understood process involving inflammation or ischemia of the retinal vessel wall, may occur in association with a systemic process, although it can also be isolated to the retina. Because of the limited ability to perform histopathological studies on retinal vessels, there is no gold standard for diagnosis. Thus, there is utility in creating a classification system for retinal vasculitis and improving diagnostic strategies for this disease. We provide a framework for understanding retinal vasculitis based on size, location, and etiology. We hope that this information can be implemented in the clinical setting to provide some diagnostic strategies for this often confusing entity.     

Neovascular glaucoma as a complication of retinal vasculitis in Crohn disease

PURPOSE: To report a case of neovascular glaucoma as a complication of retinal vasculitis in Crohn disease. METHODS: Case report with fluorescein angiogram. RESULTS: A 62-year-old man with biopsy-proven Crohn disease presented with bilateral uveitis, bilateral iris new vessels, and neovascular glaucoma in the left eye. Fluorescein angiography revealed signs of retinal vasculitis and capillary nonperfusion in both eyes. CONCLUSION: Crohn disease may be associated with retinal vasculitis and, thus, neovascular glaucoma. A satisfactory result can be achieved by using corticosteroids to control the retinal vascular inflammation, by applying panretinal photocoagulation and by controlling the increased intraocular pressure surgically.     

Unilateral retinal vasculitis,branch retinal artery occlusion and subsequent retinal neovascularization in Crohn's disease

Purpose: To report on a case of Crohn's disease and unilateral retinal vasculitis, branch retinal artery occlusion and subsequent retinal neovascularization. Methods: We examined a 38-year-old woman with severe left visual loss and biopsy-proven Crohn's disease diagnosed four years prior to the ocular involvement. A Heidelberg scanning laser ophthalmoscope was used for fundus fluorescein angiography and indocyanine green angiography. Retinal neovascularization was detected during the follow-up. Results: Successful regression of retinal neovascularization was achieved after argon green laserpanretinal photocoagulation in addition to oral steroid and salazopyrine. Conclusion: Retinal vascular involvement is a rare ocular feature of Crohn's disease and may result in retinal neovascularization that may necessitate prompt laser photocoagulation.     

Unusual presentation of a multiple sclerosis case involving central retinal artery occlusion

The term intermediate uveitis (IU) refers to a subgroup of uveitis in which the vitreous is the site of greatest inflammation. Patients with multiple sclerosis (MS) have a greater frequency of IU compared with the general population.The IU associated with MS is characterized by the presence of pars planitis (occasionally accompanied by anterior uveitis) and the presence of peripheral retinal vasculitis in the form of periphlebitis (venous sheathing) in 6–26% of patients.We present a patient with an unusual initial presentation of MS involving central retinal artery occlusion (CRAO) in the right eye (RE).Although retinal vascular changes are asymptomatic in the majority of MS patients, the spectrum of impairment ranges from simple peripheral retina periphlebitis to the presence of peripheral occlusive retinal vasculitis in 6.5% of patients.This atypical case may represent an extreme of the spectrum of retinal vasculitis associated with demyelinating disease.     

Retinal Vasculitis Associated with Asymptomatic Gardnerella vaginalis Infection: A New Clinical Entity

Purpose: To report on 3 cases of retinal vasculitis associated with asymptomatic Gardnerella vaginalis (GV) infection. Methods: Review of 3 consecutive patients who presented with central retinal vasculitis without signs or symptoms of systemic disease. The vasculitis involved the central branches of the retinal vessels bilaterally and was steroid-dependent. Results: During treatment, patients developed clinically significant vaginitis, which the gynecologist considered to be exacerbated by the steroid treatment, leading to its withdrawal. All 3 vaginal specimens were positive for GV. Antibiotic susceptibility testing led to administration of oral ampicillin (2 g/day for 10 days), which resolved both the vaginal infection and the retinal vasculitis. Conclusions: Idiopathic retinal vasculitis can be triggered by several agents. In this case series, GV was associated with retinal vasculitis, which was resolved by oral ampicillin. GV infection may be one of a number of triggers of retinal vasculitis. Appropriate treatment and full resolution of ocular inflammation requires exclusion of possible underlying infections.     

Role of Ultra-Wide Field Imaging in the Management of Tubercular Posterior Uveitis

Purpose: To assess the utility of ultra-wide field (UWF) versus conventional fundus imaging in the management of tubercular (TB) posterior uveitis.

Methods: Twenty-two consecutive patients (33 eyes) diagnosed with TB posterior uveitis in a tertiary care center who underwent UWF fundus photography and fluorescein angiography (FA) between July 2014 and March 2015 were included. Complete clinical and imaging records of the patients were retrospectively reviewed. A circle simulating the central 75-degree field was drawn on UWF pseudocolor and fluorescein angiography images. Findings within the circle were compared with the information yielded by the complete image and its impact on patient management was noted.

Results: The clinical manifestations of posterior tubercular uveitis included retinal vasculitis (17 eyes), multifocal serpiginoid choroiditis (13 eyes), choroidal granulomas (2 eyes) and intermediate uveitis (1 eye). UWF imaging revealed additional capillary non-perfusion areas, neovascularization, active vasculitis, and peripheral choroiditis lesions in 30/33 eyes (90.9%), which influenced treatment decision in 15 eyes (45.5%).

Conclusions: UWF imaging is useful in the detection of peripheral pathologies in tubercular posterior uveitis that may influence management decisions, such as addition of immunosuppressive therapy or scatter laser photocoagulation.     

Post-Streptococcal Vasculitis

Purpose: To report a case of post-streptococcal retinal vasculitis. Design: Review of a case. Methods: Review of patient chart and flourescein angiography. Results: A 17 year old African American female presented with moderate vitritis and macular edema in the right eye and retinal vessel sheathing in both eyes. Conclusions: Although CNS vasculitis is well recognized as a post-streptococcal syndrome, this case illustrates that retinal vasculitis can also occur in this setting, and can resolve with oral steroid therapy.     

Use of Ultrasound Biomicroscopy, Indocyanine Green Angiography and HLA-B51 Testing as Adjunct Methods in the Appraisal of Behçet's Uveitis

Purpose: Behçet's uveitis is not common in western Europe and thedisease presentation is less obvious than in ``endemic'' countries such as Turkeyand Japan. This makes the diagnosis more difficult. Early diagnosis is important,as the prognosis is better if therapy is started early. New methods such as ultrasoundbiomicroscopy (UBM) and indocyanine green angiography (ICGA) can improve thecharacterisation and diagnosis of uveitis. Our purpose was to present our experiencewith these new methods as well as HLA-B51 testing in the appraisal of patients withBehçet's uveitis.Patients and Methods: Patients seen by the authors between 1997 and 2001with Behçet's uveitis or suspected Behçet's uveitis and who underwentICG angiography or UBM were included. Symptoms and signs, results of laboratorywork-up including HLA-B51 antigen testing and the delay to diagnosis, were analysed.Fluorescein and ICG angiography and UBM testing were performed according tostandard protocols used for uveitis patients and their contribution towards diagnosisand management were analysed.Results: Uveitis was non granulomatous in all patients. Fluorescein angiography showed moderate to severe diffuse retinal vasculitis compatiblewith Behçet's uveitis in all cases. HLA-B51 testing was positive in 5 of 7tested cases, being useful to orient the diagnosis. UBM contributed to thediagnosis in all five tested cases, being the determining element in 3 patients.It allowed redirection of the diagnosis from pars planitis to Behçet's in 2patients with poorly transparent media because it failed to show the typicalpars planitis deposits. In a case originally diagnosed as Behçet's it allowedcorrection of the diagnosis to pars planitis because of the presence of the typicalUBM pars plana depositis. ICG angiography allowed detection of choroidalvasculitis in all five tested cases.Conclusions: In Behçet's patients who did not present with a full-blownclinical picture, as they are often seen in non-endemic areas, UBM examinationand HLA-B51 testing were valuable additional diagnostic elements helping toredirect the diagnosis correctly and to reduce the diagnostic delay in these patients.The hitherto unknown choroidal vasculitis shown by ICG angiography in all fiveinvestigated patients indicates that choroidal involvement probably occurs in mostnewly diagnosed Behçet's patients.     

视网膜血管炎的研究进展

视网膜血管炎主要累及青壮年视网膜静脉,动脉累及较少,致盲率较高.积极防治此病意义重大.其发生可能与感染、免疫机制、氧化反应、全身性疾病等有关,通过荧光素眼底血管造影、超声、实验室检查可提高诊断正确率.针对不同的病程,采用药物、激光、玻璃体视网膜手术等治疗措施可改善视功能.就此病的定义、病因、分类、基础研究、诊断及治疗等方面的最新进展进行综述.     

A clinical approach to the diagnosis of retinal vasculitis

Retinal vasculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and is confirmed with the help of fundus fluorescein angiography. Active vascular disease is characterized by exudates around retinal vessels resulting in white sheathing or cuffing of the affected vessels. In this review, a practical approach to the diagnosis of retinal vasculitis is discussed based on ophthalmoscopic and fundus fluorescein angiographic findings.     

分频幅去相干血管成像

相干光断层扫描血管成像(optical coherence tomography angiography,OCTA)通过比较同一位置的不同扫描,去除不变的结构,即可在不使用造影剂的情况下观察到有红细胞流动的视网膜和脉络膜血管结构.目前,一种新的运算方法——分频幅去相干血管成像(split-spectrum amplitude decorrelation angiography,SSADA)的应用,大大改善了OCTA的信噪比.与其他OCTA不同的是,SSADA OCT可获取与OCT光波平行或垂直的血流信号,不依赖多普勒角,并且图像噪音、组织运动等噪音较小.目前SSADA OCT已越来越多地应用于多种眼部疾病中,如脉络膜新生血管性疾病、糖尿病视网膜病变,视网膜血管阻塞等,在这些疾病的诊断、随访中均有重要意义.