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Retinal vasculitis.

Retinal vasculitis represents a group of diseases characterized by inflammation affecting the retinal vasculature. It is an uncommon disorder that may occur as an isolated disease or more commonly in association with other ocular diseases or a variety of systemic diseases. With a wide variety of disease associations, a search for an underlying etiology should be undertaken based on a meticulous history, review of systems, and physical examination. The laboratory evaluation of patients with retinal vasculitis is an essential component of the work-up to facilitate detection of any underlying disease or to establish a limited differential diagnosis. The management of infectious causes of retinal vasculitis consists of antimicrobial therapy while noninfectious retinal vasculitis is managed with corticosteroids and/or immunosuppressive agents. Because retinal vasculitis is an uncommon disease, there are only a limited number of publications over the past year related to this topic.

Eye Diseases↗

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.

Diagnosis, Differential↗

The pathology and pathogenesis of retinal vasculitis.

Retinal vasculitis is a rare, but potentially blinding intraocular inflammatory condition with diverse aetiology. Although commonly idiopathic, it has a strong association with systemic inflammatory diseases known to involve other areas of the central nervous system, most notably Behcet's disease, sarcoidosis, systemic lupus erythematosis and multiple sclerosis. This article describes the clinicopathologic features of retinal vasculitis and its visually damaging sequelae, reviewing available human histopathologic studies and work with experimental models to discuss the pathogenesis and immunopathology. Evidence indicates that noninfective retinal vasculitis is an autoimmune condition that may be induced by antecedent infection with microbes cross-reacting with putative autoantigens, influenced by genetic susceptibility of both HLA associations and cytokine polymorphisms. The growing understanding of the cellular mechanisms involved in the effector immune response is already providing a rationale for more specific therapeutic approaches.

Humans↗

Serum levels of chemokines correlate with disease activity in patients with retinal vasculitis.

Retinal vasculitis (RV) is characterised pathologically by migration of leucocytes across the blood-retinal barrier leading to oedema and photoreceptor cell dysfunction. Chemokines are a family of small molecules involved in leucocyte migration. In this study, levels of chemokines were measured in serum from patients with RV and correlated with disease activity and drug treatment. Serum samples (n= 100; 25 active, 75 inactive) were obtained from 50 patients with RV, and levels of the chemokines MIP-1alpha, macrophage inflammatory protein-1beta (MIP-1beta) and monocyte chemoattractant protein-1 (MCP-1) were measured by ELISA. For longitudinal analysis levels of the same chemokines were measured in six consecutive serum samples from 10 of the above patients. Chemokine levels were correlated with disease activity and current drug treatment for each sample. Sera from 20 healthy individuals were used as control samples. Serum levels of MIP-1beta were significantly raised in patients with RV, whether active or not, compared to healthy controls (P= 0.04). Levels of MIP-1beta and MCP-1 correlated with disease activity in some patients and with prednisolone levels in patients on this treatment alone. MIP-1alpha levels were not detectable in all samples but were present in significantly more samples from patients with active or inactive RV compared with healthy controls. Serum levels of the chemokines MIP-1beta and MIP-1alpha, but not MCP-1 were raised in patients with retinal vasculitis. Longitudinal analysis suggested that MIP-1beta and MCP-1 levels were controlled by drug treatment, particularly prednisolone. These data demonstrate that chemokines are involved in the pathogenesis of RV and may act as novel therapeutic targets.

Chemokine CCL2↗

[Retinal vasculitis].

Retinal vasculitis is an inflammatory permeability disorder of the retinal vascular wall affecting chiefly the veins and capillary vessels. It is diagnosed at ophthalmoscopy which demonstrates vascular sheathing and even better at fluorescein angioscopy which shows the colouring agent diffusing from the vessels. As regards its aetiology and treatment, retinal vasculitis is disconcerting. Except in those cases where a systemic disease can be blamed, the condition is isolated, usually chronic and often recurrent. It may result in visual impairment or, exceptionally, blindness.

Fluorescein Angiography↗

[Retinal vasculitis].

Retinal vasculitis is one of the frequently cause of impair of visual acuity. The vein is involved most of time, and the artery is involved less. Sometimes, the vein and artery are involved together. The types and cause of the disease are complex. In recent years, ophthalmologists have gotten some new achievements on the study of the disease, and some arguments just like naming, diagnosing and treatment still exist. Now we review all the achievement and arguments of the naming, clinic, pathology, etiology, experiment, diagnosing, treatment of the disease.

Humans↗

Acute severe uveitis with retinal vasculitis and retinal detachment.

5 adult patients (7 eyes) with acute severe uveitis with retinal vasculitis and retinal detachment of unknown etiology were studied. Yellowish exudations which appeared in the peripheral fundus rapidly extended posteriorly, and there was a patchy perivasculitis, white sheathing and obliteration of the retinal vessels. The absorption of these yellowish exudations was followed by atrophy of the retina and the choroid. Secondary detachment of the retina then developed, and the visual acuity remained poor in most cases.

Acute Disease↗

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 laser panretinal 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.

Adult↗

Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. Retinal Vasculitis Study.

PURPOSE: The authors describe the clinical feature of ten patients with a new syndrome characterized by the presence of retinal vasculitis, multiple macroaneurysms, neuro-retinitis, and peripheral capillary nonperfusion. METHODS: The authors evaluated ten patients identified to have clinical features compatible with the syndrome of idiopathic retinal vasculitis, aneurysms and neuroretinits (IRVAN). Clinical examination findings, sequential funds photographs (when available), fluorescein angiograms, systemic investigations, response to therapy, and visual outcomes were reviewed. RESULTS: Seven eyes of four patients sustained a marked decrease in visual acuity of 20/200 or worse. Visual loss was due to a combination of an exudative maculopathy and sequelae of retinal ischemia. Capillary nonperfusion was seen in all ten patients and was severe enough to warrant panretinal laser photocoagulation in six patients. Systemic investigations were uniformly noncontributory. Oral prednisone appears to have little beneficial effects on patients with this disorder. CONCLUSIONS: Patients with IRVAN have characteristic retinal features that readily identify this syndrome. An increased awareness of this rare syndrome may help to identify sight-threatening complications at an earlier stage. The authors caution against extensive medical investigations.

Adult↗

Retinal vasculitis and vitreous hemorrhage associated with mixed connective tissue disease: retinal vasculitis in MCTD.

BACKGROUND: Retinal vasculitis associated with mixed connective tissue disease (MCTD) has rarely been reported in the literature. We describe a rare case of a patient with serious retinal vasculopathy and vitreous hemorrhages associated with MCTD. CASE: A 53-year-old woman with MCTD who has had recurrent retinal vasculitis and vitreous hemorrhage in the right eye since 2000 presented with blurred vision in the right eye in April 2003. The best-corrected visual acuity was counting finger in the right eye. The patient was clinically diagnosed as having retinal and vitreous hemorrhages and retinal infarction. Prednisolone was administered orally in a daily dose of 20 mg. RESULTS: The retinal and vitreous hemorrhage improved in 7 days. The best-corrected visual acuity improved to more than 20/20 on prednisolone alone. CONCLUSION: MCTD can be associated with retinal vasculitis and vitreous hemorrhage which in our case responded well to systematic low doses of corticosteroid therapy.

Adult↗

Extensive dynamics in location, shape, and size of aneurysms in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis.

PURPOSE: To describe an unusual case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome with rapid dynamics in the number and appearance of the aneurysms. DESIGN: Observational case report. METHODS: Clinical and angiographic data of the patient were reviewed. RESULTS: In the course of only 6 months, preexisting retinal aneurysms resolved while new ones appeared. Changes were observed in the shape and size of preexisting lesions. The resolution of lesions in eyes previously untreated by laser is reported for the first time. CONCLUSIONS: Vascular lesions in IRVAN syndrome may show an unusually rapid turnover. The resolution of aneurysms is a part of the natural course of the disease and may occur without previous retinal laser photocoagulation.

Adult↗

[Conservative therapy of isolated retinal vasculitis].

Isolated retinal vasculitis (IRV) is a form of retinal vasculitis without other types of eye inflammation or systemic vasculitis. Treatment of IRV is a difficult problem. We report the results of treatment of 43 IRV patients (25 men and 18 women, mean age 32.5 +/- 5.5 years with a range of 22-42 years). The main therapy for IRV were corticosteroids. They were used by pulse therapy, orally and locally (periocular injections). Improvement of visual acuity was more pronounced in patients treated by pulse therapy (0.35 +/- 0.15) than in those treated orally (0.16 +/- 0.06, p = 0.0132) and parabulbarly (0.06 +/- 0.12, p = 0.0009). Resorption of retinal perivascular exudate was sooner achieved by steroid pulse therapy than by oral treatment (8.1 +/- 2.2 days vs. 17.9 +/- 2.8 days, p < 0.0000) or periocular injection of steroids (30.9 +/- 7.1 days, p < 0.0000). Prednisolone (10-20 mg) was prescribed for 4-12 months in order to prevent IRV recurrences. Steroid-resistant patients were treated with cyclosporin A (5 mg/kg). The treatment was supplemented by fibrinolytics, angioprotectors, and antioxidants.

Acute Disease↗

Interferon-beta and adhesion molecules (E-selectin and s-intracellular adhesion molecule-1) are detected in sera from patients with retinal vasculitis and are induced in retinal vascular endothelial cells by Toll-like receptor 3 signalling.

Retinal vasculitis is a major component of ocular inflammation that plays a role in retinal tissue damage in patients with idiopathic uveitis and Behçet's disease. Here we show that type 1 interferons (IFN alpha/beta) were not detected in sera from normal individuals but were identified in up to 46% of the sera from retinal vasculitis patients. The predominant form of IFN observed was IFN-beta, which was detected in 39% of Behçet's disease patients and 47% of idiopathic uveitis patients. Seven patients whose sera contained IFN-beta were monitored prospectively. IFN-beta was shown to be present for 6-12 months in all seven of the sera samples tested. Furthermore, the adhesion molecule profile identified in this study was strikingly different when Behçet's and uveitis patient sera were compared to sera from normal controls. Sera from Behçet's disease patients contained significantly elevated levels of the soluble adhesion molecules, sE-selectin and s-intracellular adhesion molecule-1 (sICAM-1), whereas sera from patients with idiopathic uveitis contained significantly increased sE-selectin. In vitro studies evaluating the cell source of these cytokines revealed that polyriboinosinic polyribocytidylic acid (poly I:C) activated retinal vascular endothelial cells produce sE-selectin, sICAM-1 and IFN-beta. Production of these molecules was inhibited by pretreatment with anti-Toll-like receptor 3 (TLR-3) antibody. In conclusion, IFN-beta, sE-selectin and sICAM-1 are elevated in patients with retinal vasculitis and are induced in retinal vascular endothelial cells in vitro by activating the innate immune system through TLR-3. Further analysis of innate immune signalling may prove to be a novel target for future studies on pathogenic mechanisms and therapeutic approaches in retinal vasculitis.

Acute Disease↗

Retinal vasculitis--a primer.

Retinal vasculitis is a diagnosis that is generally suggested by an ophthalmologist. Frequently patients with the disorder are referred to nonophthalmologists for further diagnostic evaluation or treatment. The criteria for defining vasculitis differ greatly between ophthalmologists and other physicians. To facilitate collaboration between ophthalmologists and their colleagues, we have sought to clarify the term "retinal vasculitis" by discussing its subcategories, the potential role of antiphospholipid antibodies, and the etiology of retinal vasculitis. We offer guidelines for evaluating the disorder and treating patients.

Humans↗

Retinal vasculitis in Lyme borreliosis.

We observed retinal vasculitis in seven patients with clinical and serologic evidence of Borrelia burgdorferi infection. Three patients presented with abrupt loss of vision due to acute retinal vasculitis. Funduscopy demonstrated engorged veins, hemorrhages, perivenous infiltrates and retinal white spots. Fluorescein angiography showed leakage from the veins, from the white spots and from the optic disc. Moreover arterial occlusions were observed in two patients. Four patients had signs of chronic uveitis with vitritis, cystoid macular oedema and retinal vasculitis, which was associated with neovascularization and vitreous hemorrhage in one patient, and with optic neuritis in another patient. Six patients received antibiotic treatment and three patients received systemic corticosteroids. Marked improvement in the three acute retinal vasculitis cases occurred within several weeks, the fundus changes disappeared in another few months, and no recurrences were observed. The final visual acuity was excellent in these patients, although optic disc pallor and visual field loss persisted in one case. In the four patients with chronic uveitis visual blurring improved following antibiotic treatment and the retinal vasculitis and vitritis slowly regressed. The proliferative retinopathy of one patient required panretinal laser treatment.

Adult↗

Retinal vasculitis.

Inflammation of the retinal vasculature may occur as a manifestation of an infectious disease, as part of a systemic inflammatory disease, or as part of an isolated retinal disease, and can result in severe and permanent visual loss. The search for a cause in patients with retinal vasculitis requires appropriate systemic evaluation and testing. Discrimination between the infectious or noninfectious etiology of retinal vasculitis is important because the treatment is different.

Fluorescein Angiography↗

Human T-cell lymphotropic virus type 1-associated retinal vasculitis in children.

PURPOSE: To describe predominant retinal vasculitis in children carrying human T-cell lymphotropic virus type 1 (HTLV-1). METHODS: The authors examined clinical records of patients with HTLV-1-associated uveitis between 1987 and 2001 in Kagoshima University Hospital and reviewed cases of extensive, smoldering retinal vasculitis. RESULTS: Three previously healthy teenagers noted mild visual symptoms and presented with extensive sheathing of retinal vessels, complicated by mild anterior segment inflammation. The retinal vascular disease responded poorly to systemic corticosteroids, had a smoldering course with persistent sheathing of retinal vessels, and eventually resulted in diffuse chorioretinal degeneration. Results of laboratory studies were unremarkable except for the presence of serum antibodies to HTLV-1. One patient developed HTLV-1-associated myelopathy 11 years after the onset of ocular disease. CONCLUSIONS: The retinal vasculitis differed from the retinal vascular changes commonly seen in HTLV-1-associated uveitis. The authors suggest a clinical disease HTLV-1-associated retinal vasculitis that affects young HTLV-1 carriers, characterized by smoldering retinal vasculitis with ultimate retinal degeneration.

Adolescent↗