Diagnosis Of Retinal Vasculitis Research Articles

Clinical course of choroidal neovascular membrane in West Nile virus chorioretinitis: a case report

BackgroundThis report describes the clinical course of choroidal neovascular membrane (CNV) in West Nile virus-associated chorioretinitis.Case presentationA 28-year-old Italian woman was referred to our institution because of reduced visual acuity in the left eye dating back 4 months. A diagnosis of retinal vasculitis in the right eye and chorioretinitis with CNV in the left eye was made. A complete workup for uveitis revealed positivity only for anti-West Nile virus immunoglobulin M (IgM), while immunoglobulin G (IgG) was negative. Whole-body computed tomography and nuclear magnetic resonance imaging of the brain were also negative. Therefore, the patient was treated with a combination of oral prednisone (starting dose 1 mg/kg per day) and three intravitreal injections of bevacizumab 1.25 mg/0.05 ml, 1 month apart. Fourteen days from starting corticosteroid therapy and after the first intravitreal injection, the patient experienced increased visual acuity to 0.4. Response to therapy was monitored by clinical examination, ocular coherence tomography (OCT), OCT angiography and retinal fluorescein angiography. Three months later, resolution of CNV in the left eye was achieved and no signs of retinal vasculitis were detected in the right eye, while serum IgM for West Nile virus turned negative and IgG positive.ConclusionCNV may be a complication of West Nile virus-associated chorioretinitis, and only subclinical retinal vasculitis may also be found even in non-endemic regions.

Retinal Vasculitis and Intraocular Inflammation after Intravitreal Injection of Brolucizumab

To evaluate features and outcomes of eyes with retinal vasculitis and intraocular inflammation (IOI) after intravitreal injection (IVI) of brolucizumab 6 mg/0.05 ml for treatment of neovascular age-related macular degeneration. Retrospective case series. Fifteen eyes from 12 patients identified from 10 United States centers. Review of patient demographics, ophthalmologic examination results, and retinal imaging findings. Baseline and follow-up visual acuity (VA), prior anti-vascular endothelial growth factor (VEGF) injections, clinical presentation, retinal findings, fluorescein angiography results, and treatment strategies. The number of previous anti-VEGF IVIs ranged between 2 and 80 in the affected eye before switching to brolucizumab. Retinal vasculitis and IOI were diagnosed at a mean of 30 days after brolucizumab IVI. Mean VA before brolucizumab IVI was 0.426 logarithm of the minimum angle of resolution (logMAR; Snellen equivalent, 20/53) and VA at diagnosis of retinal vasculitis was 0.981 logMAR (Snellen equivalent, 20/191; range, 20/25-20/1600; P= 0.008). All affected eyes showed IOI with variable combinations of focal or elongated segmental sheathing and discontinuity of small and large retinal arteries, sclerotic arteries, regions of vascular nonperfusion, cotton-wool spots, Kyrieleis plaques, irregular venous caliber with dilated and sclerotic segments, perivenular hemorrhages, and foci of phlebitis. Fluorescein angiography revealed delayed retinal arterial filling, retinal vascular nonperfusion, and variable dye leakage from affected vessels and the optic nerve. Systemic evaluation for embolic causes was unrevealing in 2 patients, and 3 patients showed negative laboratory assessment for uveitis. Treatment consisted of various combinations of corticosteroids (systemic, intravitreal, and topical), and 2 eyes underwent vitrectomy without improvement in vision. After a mean follow-up of 25 days, mean VA was 0.833 logMAR (Snellen equivalent, 20/136), which was reduced compared with baseline (P= 0.033). Retinal vasculitis and IOI after brolucizumab IVI are characterized by variable occlusion of large or small retinal arteries, or both, and perivenular abnormalities. It may span from peripheral vasculitis to occlusion of large retinal arteries around the optic nerve or macula with severe vision loss. A high index of suspicion is required because vitreous cells may obscure visualization of retinal details.

Retinal vasculitis with Chronic Recurrent Multifocal Osteomyelitis: a case report and review of the literature

BackgroundConcurrent presentation of retinal vasculitis with mixed sclerotic and lytic bone lesions is rare.Case presentationWe present the case of a 37-year old woman with a several year history of episodic sternoclavicular pain who presented for rheumatologic evaluation due to a recent diagnosis of retinal vasculitis. We review the differential diagnosis of retinal vasculitis, along with the differential diagnosis of mixed sclerotic and lytic bone lesions. Ultimately, bone marrow biopsy confirmed diagnosis of chronic recurrent multifocal osteomyelitis (CRMO). Concurrent presentation of CRMO with retinal vasculitis is extremely rare but important to recognize. The patient demonstrated clinical response to prednisone and tumor necrosis factor-alpha inhibition (TNF-i).ConclusionThis case reports and unusual presentation of CRMO spectrum disease involving the sternum and sternoclavicular joint with concurrent retinal vasculitis.

OPTICAL COHERENCE TOMOGRAPHY WITH ANGIOGRAPHY IN BEHÇET UVEITIS

Purpose: Behçet uveitis is a cause of retinal vasculitis, with fluorescein angiography being the gold-standard diagnostic exam. Optical coherence tomography with angiography (OCT-A) is a non-invasive exam that analyzes macular retinal circulation with high resolution, and therefore, could be a useful diagnostic exam in these patients.Material and methods: Analysis of 14 eyes of 8 patients with Behçet uveitis from our institution that were examined by OCT A (AngioPlex®, Zeiss). Macular morphology, area, perimeter and circularity of the foveal avascular zone, as well as central internal and global macular vascular density were evaluated. We compared our results with healthy controls.Results: Mean visual acuity in the patient’s subgroup was 0,84±0,25 and 3 eyes had previous diagnosis of retinal vasculitis with fluorescein angiography. Morphologically we found attenuation of the superficial and deep capillary plexus in 8 eyes, and in 1 of these eyes atrophy of the internal retinal layers was also found in a localized territory. In the foveal avascular area, no statistically significant changes were observed in its area and perimeter (p=0.804 and p=0.380 respectively), although we did find significant changes in its circularity (p=0.027). Vascular density was significantly altered in the internal (p=0.023) and global (p=0.028) macular parameters.Conclusion: We found changes in the macular microcirculation in patients with Behçet uveitis, even in the absence of angiographic changes. Future studies with more patients are necessary to understand the clinical significance of these changes.

Interferon-gamma assay in tuberculous uveitis

Tuberculous uveitis can be an elusive diagnosis and skin testing can yield equivocal results particularly in subjects who have received previous Bacille Calmette Guerin (BCG) vaccination. We present a case...

RETINAL VASCULITIS ASSOCIATED WITH SYSTEMIC DISEASE: Behçet's Disease, Sarcoidosis, Inflammatory Bowel Disease, and Others

Retinal vasculitis is a nonspecific immunologic reaction of retinal arterioles or venules to various antigenic stimuli that may occur in many different systemic and local disease states. 33 There are no clear-cut clinical criteria for the diagnosis of retinal vasculitis. It may be defined, 26 however, to be present when there are vascular leakage and staining of vessel walls on fundus fluorescein angiography (FFA), with or without the 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 intraretinal edema, or infiltrates, it is more likely to be inflammatory in origin. 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. The approach to retinal vasculitis should aim at assessing the effects of vascular inflammation on the retina and visual function, and also at determining the underlying cause. Retinal vasculitis may be a major feature in certain diseases, and in others occur as a secondary phenomenon. 86 Toxoplasmosis and cytomegalovirus retinopathy (fig. 1) are examples of the latter, in which treatment of the primary pathology will result in resolution of the vasculitis. It is thus important to search the fundus for any localizing cause. If absent, one should then search for any underlying systemic disease, with the help of an internist. Retinal vasculitis may be associated with infections, malignancies, or multisystem inflammatory disorders. 86 The more commonly associated systemic diseases include Behcet's disease (BD) and sarcoidosis. 72,111 In most cases, however, the retinal vasculitis remains idiopathic and confined to the eye, 72,111 and subjecting a patient to extensive diagnostic evaluation is often unrewarding; false-positive diagnostic test results have been found in 21% of patients. 25 The workup for patients should depend on a differential diagnosis arrived at following a thorough review of systems and careful physical examination. The investigations for a patient without systemic symptoms include an FFA to determine whether the vasculitis is occlusive or nonocclusive, complete blood count, erythrocyte sedimentation rate (ESR), urinalysis, fluorescent treponemal antibody absorption test (FTA-ABS), rapid plasma reagent (RPR), and a chest roentgenogram. 25 An attempt at differentiating whether the arteries or veins are predominantly involved may aid in the differential diagnosis. 102

FROSTED BRANCH ANGIITIS: Idiopathic Retinal Vasculitis, Aneurysms, Neuroretinitis, and Acute Multifocal Hemorrhagic Retinal Vasculitis

In the diagnosis of a systemic vasculitis, a physician starts with the patient history and examination. Later appropriate laboratory tests are ordered. After these are reviewed, it is common to perform a biopsy and use histopathologic examination to help diagnose and classify the vasculitis. The diagnosis of a retinal vasculitis also starts with a history and examination. With the retinal vasculitides clinicians have the advantage of actually being able to see the inflamed vessel. The vasculitis may involve any or all elements of the vascular circuit, including the arterioles, capillaries, and venules. The proper diagnosis of retinal vasculitis frequently depends on pattern recognition, because many conditions cannot be confirmed by laboratory testing. In addition, biopsy of the intraocular is impractical or overly invasive. The retinal vasculitides are usually split into two large groups: those with a primarily localized ocular problem and those with a systemic disease that may also have retinal vasculitis as a component. On occasion a patient with a systemic disease, such as sarcoidosis or leukemia, may present with a retinal vasculitis as the first sign of disease. Many patients with a retinal vasculitis as part of systemic disease have a known systemic diagnosis on presentation to the ophthalmologist. The remaining patients with retinal vasculitis have a localized ocular problem. Some of these patients will have nonspecific inflammation of retinal vessels. Other patients may have additional signs that may help in classifying the vasculitis. The identification of these differentiating features relies on pattern recognition. This article discusses three entities that are diagnosed primarily by their ophthalmoscopic appearance. For the most part, there are no supportive laboratory tests or biopsies available to help establish or confirm the diagnosis. Some patients present with an incredible sheathing of the retinal vessels, to the extent that the blood column is obsured by a coat of perivasculitis. In 1976 Ito and associates 14 examined a 6-year-old boy who had bilateral uveitis with profound sheathing of the retinal vessels. Based on the opthalmoscopic appearance, and not on the pathophysiology or histopathology, the retinal vasculitis was called frosted branch angiitis. Since then a number of cases have been reported in Japan, the United States, and elsewhere with additional features. In addition, some patients have had frosted branch appearance secondary to an ocular or systemic disease. A classification system of frosted branch angiitis based on clinical course, proposed cause, and treatment is presented.

A point prevalence study of 150 patients with idiopathic retinal vasculitis: 1. Diagnostic value of ophthalmological features.

This paper describes the ophthalmological features of 150 patients with idiopathic retinal vasculitis, 67 of whom had isolated retinal vasculitis (RV) and 83 had RV associated with systemic inflammatory disease (RV + SID). The diagnosis of retinal vasculitis was made by ophthalmoscopy and fluorescein angiography, and patients with any identifiable cause (infection, ischaemia, or malignancy) were excluded from the study. Patients with isolated RV tended to have peripheral vascular sheathing, macular oedema, and diffuse capillary leakage. Those with RV accompanying Behçet's disease often had branch vein retinal occlusions and retinal infiltrates together with macular oedema and diffuse capillary leakage; the retinal infiltrates were pathognomonic for Behçet's disease. In sarcoidosis the retina typically showed features of periphlebitis associated with focal vascular leakage. Patients with uveomeningitis, multiple sclerosis, arthritis, or systemic vasculitis showed diffuse retinal capillary leakage associated with a mixture of the other features. Poor visual function was particularly associated with macular oedema and branch vein retinal occlusion, while the retina appeared to 'withstand' the impact of vascular sheathing, periphlebitis, or neovascularisation alone. Within the limitations of a point prevalence study it was concluded that different patterns of retinal vasculitis occur in different systemic inflammatory diseases, and that in isolated retinal vasculitis there is a particular association between peripheral vascular sheathing, macular oedema, and diffuse capillary leakage. In Part 2 we describe the results of examining the sera of these patients for the presence of antiretinal antibodies and circulating immune complexes.