Idiopathic Retinal Vasculitis Research Articles

Long-Term Course in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis

We report the long-term course and multimodal imaging findings of a patient with IRVAN syndrome who was successfully treated that developed a branch retinal artery occlusion and demonstrated virtually complete resolution of aneurysmal dilatations.

Enhanced S-cone syndrome (clinical case)

A case of enhanced S-cone syndrome misdiagnosed as idiopathic retinal vasculitis is presented. Etiology, pathogenesis and clinical features are described below.

A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

We aimed to analyze the clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Furthermore, we aimed to correlate the number and location of retinal aneurysms with the size of retinal non-perfusion area and neovascularization. Six patients with IRVAN syndrome (1 male and 5 females, age 5-38years) were enrolled in this study. Fundus fluorescein angiography (FFA) was used to determine the total number of retinal aneurysms, number of aneurysms within the first branch of the retinal artery, minimum distance between the non-perfusion margin and the optic disc, and the number of retinal aneurysms in each quadrant, as well as the type of neovascularization. The size of the non-perfusion area was positively correlated with the total number of retinal aneurysms, the number of aneurysms within the first branch of the retinal artery, and the number of retinal aneurysms in each quadrant (P < 0.05). During the 5-year follow-up, one patient exhibited a dynamic change in the number and location of retinal aneurysms. In IRVAN syndrome, the number and location of retinal aneurysms correlate with the size of retinal non-perfusion area and type of neovascularization.

Long-term outcomes in a series of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome

ObjectiveIdiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare entity with a potentially poor visual prognosis. Our objective is to review the clinical presentation and long-term outcomes of patients with IRVAN syndrome. DesignThis is a retrospective case series. MethodsWe reviewed the charts of all the patients diagnosed with IRVAN syndrome at our tertiary care centre from 2002 to 2015. ResultsWe included the long-term clinical outcomes of 7 eyes (5 patients) diagnosed with IRVAN syndrome. After a mean follow-up of 84.9 months, best-corrected visual acuity was 20/40 or better in the majority of eyes (70%). Four (57.1%) patients had systemic conditions, namely, multiple sclerosis, ischemic stroke, and positive antiphospholipid titres. All eyes were treated with laser photocoagulation. Four (40%) eyes received adjunctive intravitreal bevacizumab injections. ConclusionIRVAN is an important diagnosis for clinicians to recognize. When treated in a timely manner, long-term visual outcomes can be favourable.

Ultra-widefield angiography in the diagnosis and management of uveitis

PURPOSE:The aim of this study is to evaluate the clinical use of ultra-widefield angiography (UWFA) in uveitis.MATERIALS AND METHODS:We showed the UWFA and traditional 55° fluorescein angiography (FA) of four cases with Eales' disease, idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome, Behçet's disease, and idiopathic panuveitis in a tertiary medical center. Comparison of UWFA with traditional 55°FA in the diagnosis and management of uveitis and review of the literature is also presented.RESULTS:Ultra-widefield FA revealed far-peripheral vascular imaging. This information would not have been easily obtained from traditional FA. With more information of the peripheral retina, it may facilitate appropriate diagnosis and monitoring, and the management may be revised.CONCLUSION:University of Western Australia played an important role to confirm the diagnosis of uveitis and to determine the corresponding treatment.

Multimodal imaging questions etiology of idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome (IRVAN syndrome)

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a rare entity of unknown etiology for which many hypotheses have been proposed with inflammation being the most commonly accepted hypothesis. We report cases of a 9 year old girl and a 22 year old male patient with diagnosis of IRVAN syndrome. The conclusions drawn from the clinical examination and multimodal imaging including optical coherence tomography angiography of the patients are discussed. Our conclusions and interpretation point towards IRVAN being a developmental vascular anomaly rather than a consequence of inflammation. It is thus proposed to be renamed as Idiopathic retinal arteriolar aneurysm syndrome (IRAA).

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: clinical perspectives.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome typically affects young, healthy individuals. Despite the dramatic fundus appearance seen in this syndrome, these patients are usually asymptomatic. The syndrome includes peculiar vascular abnormalities in the form of multiple aneurysmal dilatations seen along retinal arterioles and optic nerve-head arterioles, which are best appreciated on fluorescein angiography. Neuroretinitis and retinal vasculitis are seen in all patients, and manifested by staining of the optic nerve head and diffuse leakage from vessels, mainly arterioles, on fluorescein angiography. The devastating vision-threatening outcomes of this syndrome include exudative retinopathy and extensive peripheral retinal nonperfusion areas, which can eventually lead to neovascularization. This review summarizes current knowledge on the variable clinical aspects of this disease, highlighting diagnostic and treatment strategies.

Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis (IRVAN): case series of three patients with multimodal imaging

PurposeTo describe the clinical presentation, disease progression, treatment and complications of IRVAN.MethodsRetrospective review of three patients with a diagnosis of IRVAN seen at the Birmingham and Midland Eye Centre between 2010 and 2017. Multimodal imaging investigations included wide‐field fluorescein angiography, optical coherence tomography, and indocyanine green angiography.ResultsA total of 6 eyes from 3 patients with bilateral disease were included. All six eyes were treated with panretinal photocoagulation (PRP). One eye received a dexamethasone implant for refractory macular oedema and exudation. One eye was treated with Avastin injections for persistent optic disc neovascularization. All 3 patients were treated with oral steroids at some point in the course of their disease and one was subsequently immunosuppressed with mycophenolate mofetil. Visual outcomes varied. One patient maintained excellent visual acuity of 6/6 in both eyes at 72‐months follow‐up. The second patient experienced progressive visual loss from 6/9 right eye, 6/6 left eye at presentation; to 6/18 right eye, 6/60 left eye at 12 months follow‐up due to macular exudation despite aggressive PRP and intravitreal Ozurdex. The third patient deteriorated from 6/5 right eye, 6/4 left eye at presentation to 6/60 right eye at 6 months, despite aggressive PRP. He had recurrent vitreous haemorrhages in the right eye secondary to NVD despite further interventions with Avastin injections; seven years after presentation, vision remained at 6/60 right eye and 6/18 left eye.ConclusionsThe cadence of progression of IRVAN can vary greatly, despite aggressive treatment with PRP, intravitreal steroids, and anti‐VEGF agents. We observed variation in disease progression both among patients as well as within the same individual, supporting an individualized approach to therapy.

Bilateral idiopathic retinal vasculitis following coxsackievirus A4 infection: a case report

BackgroundCoxsackieviruses are members of a group of viruses called the enteroviruses, which may cause respiratory and gastrointestinal symptoms, erythema, meningoencephalitis, myocarditis, pericarditis, and myositis. Unilateral acute idiopathic maculopathy caused by coxsackievirus A16 has been associated with hand, foot, and mouth disease, but only a few reports describe retinitis associated with coxsackievirus serotype B3 or B4. We report a case of bilateral multifocal obstructive retinal vasculitis that developed after coxsackievirus A4 infection.Case presentationA 60-year-old woman was referred to our department with bilateral visual disturbance that developed following flu-like symptoms. At the initial examination, best corrected visual acuity was 20/200 in the right eye and 20/50 in the left eye. The critical flicker frequency (CFF) was 23 Hz in the right eye and 27 Hz in the left eye. Fine white keratic precipitates with infiltrating cells were presented in the anterior chamber of both eyes, and multifocal retinal ischemic lesions were observed in the macula and posterior pole of both eyes. The retinal lesions corresponded with scotomas observed in Goldmann visual field test. On spectral domain-optical coherence tomography (SD-OCT), retinal lesions were depicted as hyper-reflective regions in the inner retina layers in both eyes, and disruption of ellipsoid line in the left eye., Fluorescein angiography exhibited findings indicative of multifocal obstructive retinal vasculitis. The patient had a history of current hypertension treated with oral therapy and glaucoma treated with latanoprost eye drops. Blood test for coxsackievirus antibody titers revealed that A4, A6, A9, B1, B2, B3, and B5 were positive (titers: 8–32). Abdominal skin biopsy of necrotic tissue suggested vascular damage caused by coxsackievirus. The general symptoms improved after 6 weeks, and the multifocal retinal ischemic lesions were partially resolved with residual slightly hard exudates. Only coxsackievirus A4 antibody titer increased from 4 to 32-fold after 14 months. However, hyper-reflective regions and disruption of the inner retinal layers on SD-OCT persisted especially in the right eye, and residual paracentral scotoma was observed in the right eye.ConclusionThe present case suggests that coxsackievirus A4 causes bilateral multifocal obstructive retinal vasculitis with irreversible inner retinal damage.

English

INTRODUCTION: Retinal Vasculitis is a non specific immunologic reaction of retinal arterioles or venules to various antigenic stimuli that may occur in many different systemic and local disease states. It may be defined, however, to be present when there are vascular leakage and staining of vessel walls on fundus fluorescein angiography, with or without clinical appearance of fluffy, white perivascular infiltrates in an eye with evidence of inflammatory cells in the vitreous body or aqueous humor. Retinal vasculitis may or may not be symptomatic. Inflammation of peripheral retinal blood vessels is typically asymptomatic, but inflammation involving macular retinal blood vessels frequently causes decreased visual acuity and focal scotomas. Associated vitreous inflammatory cells can give rise to floaters. The signs of vascular sheathing or cuffing can be found in inflammatory vascular disorders as well as in ischemic vasculopathies without inflammation. These two conditions should not be confused, as the approach to the diagnosis is different. If vascular sheathing is accompanied by vitreous cells, adjacent intra retinal edema, or infiltrates, it is more likely to be inflammatory in orgin. Fundus fluorescein angiography is useful in evaluating the severity and extent of disease. Mildly inflamed vessels usually only show staining with fluorescein, whereas in more severe inflammation, dye leakage occurs as well. In occlusive vasculitis, capillary fallout is seen. If untreated, neovascularization, vitreous hemorrhage, and tractional retinal detachment may ensue. AIM OF THE STUDY: To determine the various etiological pattern of retinal vasculitis. To determine the various ophthalmoscopic features at presentation. To assess visual outcome in various etiological retinal vasculitis. MATERIALS AND METHODS: It was a prospective study of patients who attended the retina and uvea service at Ophthalmology Department Government Rajaji Hospital Madurai. All cases of Retinal Vasculitis were taken up for study, irrespective of other ocular or systemic manifestation. A standard proforma was used to collect and document all the details regarding the cases included in the study. The detailed information regarding history and specific questions about presence of possible multisystem inflammatory disorders were asked. CONCLUSION: Within the limitations of this study, examination of these 41 patients has shows, different pattern of retinal vasculitis. And it is mandatory for all cases of retinal vasculitis to be examined in detail in order to look for ocular involvement other systemic involvement and to do a complete work up of investigation procedures. This will certainly help to classify the retinal vasculitis. It is evident that when case of idiopathic retinal vasculitis are detected early and treated adequately the incidence of vision threatening complication are considerable minimized. Vasculitis associated with human Immuno deficiency virus had a worse outcome due to rapid progression despite therapy. More follow up period is necessary to describe the complications of the disease and also to analyze whether case of Idiopathic Retinal Vasculitis develop any systemic feature would lead on to a specific diagnosis.

Widefield OCT Angiography of Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis

Widefield OCT Angiography of Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis

Adaptive Optics Imaging in Retinal Vasculitis

ABSTRACTPurpose: To study the sheathing of retinal vasculitis in various systemic autoimmune diseases using adaptive optics imaging (AOI).Methods: Prospective, observational case series with six patients: Behçet disease (n = 1); systemic lupus erythematosus (n = 1); idiopathic retinal vasculitis (n = 2); granulomatosis with polyangiitis (n = 1); and Takayasu aorta arteritis (n = 1). Fundus photograph (FP), fundus fluorescein angiography (FFA) were done in all cases at presentation. Using the Image J software, perivascular sheathing and wall-to-wall diameter of the vessel involved were measured on AOI at time of presentation and on follow-up.Results: AOI was able to pick the pipe-stem sheathing in SLE and IRV(I) and parallel sheathing in rest, which correlated with FP and FFA. Moreover, the decrease and a complete resolution in the sheathing were also noted by AOI on follow-up.Conclusions: AOI can be used as an additional investigative tool for diagnosis and to monitor the disease course during the treatment.

VISUAL OUTCOME AND POOR PROGNOSTIC FACTORS IN ISOLATED IDIOPATHIC RETINAL VASCULITIS.

To describe the clinical course, visual outcome, and prognosis of isolated, idiopathic retinal vasculitis. Eighty patients (150 eyes) with isolated, idiopathic retinal vasculitis were included. Demographic data, clinical data, complications at the initial visit and during follow-up, fluorescein angiography, and optical coherence tomography findings were collected from the Massachusetts Eye Research and Surgery Institution (MERSI) database from September 2005 to February 2015. Seventy-five (93.7%) patients required treatment with immunomodulatory therapy. Of those 75 patients, 60 (75%) patients were able to achieve durable remission. Factors which were independently significant predictive of poor visual outcome were lower initial visual acuity (OR: 3.78; 95% CI: 1.75-8.16; P = 0.001), cystoid macular edema (OR: 5.54; 95% CI: 1.81-16.99; P = 0.003), and macular ischemia (OR: 5.12; 95% CI: 1.12-23.04; P = 0.036). The majority (67.25%) of our patients enjoyed a good visual outcome (most recent visit best-corrected visual acuity equal to or better than 20/40 and within one line or better from the baseline) with immunomodulatory therapy. We found that cystoid macular edema, macular ischemia, and lower best-corrected visual acuity during the first consultation visit were significant independent risk factors for poor visual outcome.

Treatment and therapeutic effect of idiopathic retinal vasculitis, aneurisms and neuroretinitis syndrome

Treatment and therapeutic effect of idiopathic retinal vasculitis, aneurisms and neuroretinitis syndrome

Vanishing retinal arterial aneurysms with anti-tubercular treatment in a patient presenting with idiopathic retinal vasculitis, aneurysms, and neuroretinitis.

BackgroundIdiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome presents with characteristic clinical manifestations such as aneurysms at arteriolar bifurcations and optic nerve and retinal vascular inflammation. Regression of such features on treatment with anti-tubercular therapy (ATT) combined with corticosteroids has not been reported in literature.FindingsA 30-year-old female with sudden painless decreased vision in the left eye was referred with a diagnosis of presumed tuberculous retinal vasculitis and a positive tuberculin skin test. Based on the clinical and angiographic features of the right eye, a diagnosis of IRVAN syndrome was made. In the left eye, the patient had vitreous hemorrhage for which pars plana vitrectomy was performed. The vitreous sample was positive for Mycobacterium tuberculosis using multiplex polymerase chain reaction, and the patient was started on standard four-drug ATT and oral corticosteroids. At 6-month follow-up, vanishing of retinal arterial aneurysms was observed.ConclusionsThe pathogenesis of IRVAN syndrome is uncertain. One of the postulates is that the features of arterial aneurysms and other retinal vascular alterations occur secondary to acquired inflammatory reaction. We hypothesize that IRVAN syndrome may be a morphological diagnosis possibly associated with various entities, one of which could be ocular tuberculosis. It may be prudent to rule out intraocular tuberculosis in cases labeled as IRVAN syndrome in an endemic population.

Association of mycobacterium tuberculosis in the causation of Eales’ disease: An institutional experience

Background: Eales’ disease is an idiopathic retinal vasculitis characterized by retinal inflammation, ischemia, and neo-vascularisation. It frequently causes massive vitreous haemorrhage and retinal detachment leading to blindness. Although the exact etiology is unknown, this condition is considered to be a consequence of hypersensitivity reaction to tubercular protein due to previous Mycobacterium tuberculosis (M. tuberculosis) infection. This study is aimed at the detection of association of M. tuberculosis in patients with Eales’ disease. Materials and Methods: A prospective case-control study was undertaken in 65 clinically diagnosed cases of Eales’ disease. Patients with proliferative diabetic retinopathy, neo-vascular proliferation, macular oedema, premacular fibrosis and tractional retinal detachment were taken as controls. M. tuberculosis DNA was detected (MPT64 gene by polymerase chain reaction, PCR) in patients with Eales’ disease. Clinical symptoms along with tuberculin skin test (TST) and erythrocyte sedimentation rate (ESR) were used as gold standard for comparing results of PCR. Result: PCR positivity was found in 12 (38.7%) patients with Eales’ disease. The PCR positivity was significantly associated with the patients with high TST reading and high ESR values. Conclusion: Patients with a high TST reading and ESR value and a positive PCR in vitreous samples have a high likelihood of having M. tuberculosis as an etiology.

Optical coherence tomography features in idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome

Optical coherence tomography features in idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome

Combination Anti-VEGF and Corticosteroid Therapy for Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome

Vision loss associated with the idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome most commonly occurs from macular edema or complications related to neovascularization. The authors present a case of advanced IRVAN associated with a massive exudative response characterized by peripheral retinal telangiectasias, exudative retinal detachment, and macular edema with lipid maculopathy. The patient was managed successfully with visual acuity from hand motion to 20/150 using a combination of local corticosteroids, intravitreal bevacizumab, panretinal photocoagulation, and eventually pars plana vitrectomy for progressive vitreomacular traction. VEGF- and non-VEGF-mediated mechanisms appear to be involved in the pathogenesis of IRVAN given the efficacy of combination therapy. [ophthalmic surg lasers imaging retina. 2013;44:599-602.].

Dexamethasone Intravitreal Implant for Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis

To present the short-term favorable clinical results with the dexamethasone intravitreal implant in a patient with florid idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome (IRVAN). A 26-year-old man presented with significant bilateral deterioration of vision due to vitreous hemorrhage and neuroretinitis with a background of vasculitis and neovascularization. The patient was initially treated with high doses of oral steroids (80 mg prednisolone), which were gradually tapered, and also received extensive argon laser photocoagulation in ischemic areas in both eyes. Despite vigorous treatment and an initial positive response to treatment, pars plana vitrectomy was eventually needed to address the recurrent vitreous hemorrhages in the left eye. Consequently, visual acuity improved from 0.1 to 0.2 (Snellen) and there was no relapse of vitreous hemorrhage. Persistent macular edema was noted, however, and it was decided to treat with a dexamethasone 0.7 mg intravitreal implant. Following the dexamethasone implant OS, visual acuity improved significantly from 0.2 to 0.5 (Snellen), the patient reported much less distortion, and there was marked reduction in central retinal thickness from 467 to 234 microns. The patient remains in remission without any exudation in the macula at 4 months follow-up. Dexamethasone 0.7 mg intravitreal implant appears to be a safe and effective solution in the treatment of macular edema in patients with IRVAN syndrome and could possibly be a treatment option for other cases of inflammatory induced macular edema.

Adaptive optics imaging of retinal vasculitis

AbstractPurpose To document perivascular infiltrates in the retina using high resolution flood illumination adaptive optics (AO) imaging in patients with retinal vasculitis.Methods The charts of five subjects with retinal vasculitis showing evidence of perivascular infiltrates by means of AO imaging (rtx1 camera, Imagine Eye, Orsay, France) were reviewed.Results Diagnoses included primary idiopathic retinal vasculitis (n=1), Lyme’s disease (n=2), TB related retinal vasculitis (n=1) and idiopathic retinitis, vasculitis and aneurysm (IRVAN; n=1). Using AO imaging, perivascular infiltrates appeared as discrete, linear areas of opacification along the veins, often surrounding the venous narrowing. AO imaging was more sensitive that either fundus photographs or fluorescein angiography for their detection. Follow‐up examinations (n=3) showed a changing pattern of narrowing of the vessel lumen, and eventual disappearance of these perivascular infiltrates. In the IRVAN case, inflammatory infiltrates were found around arterioles and veins as well, suggesting an inflammation induced‐phenomenon.Conclusion AO imaging has a higher sensitivity than fundus photographs or fluorescein angiography to detect perivascular inflammatory infiltrates. Vascular inflammation is strongly linked to changes in vascular lumen diameter. AO imaging may have a particular interest when diagnosis and following subjects with retinal vasculitis.