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[Adamantiades-Behcet's disease].

Adamantiades-Behcet's disease (ABD) is a chronic recurrent vasculitis whose aetiology is still unclear. The first description goes back to B. Adamantiades und H. Behcet. The disease mainly occurs in Mediterranean, Middle and Far Eastern countries. However, it is not limited to these regions and can be found worldwide, and, as a result of increasing human mobility, is steadily becoming more significant. Clinical findings reveal recurrent oral and/or genital ulcers, arthritis and skin involvement in the form of erythema nodosum and superficial thrombophlebitis. Relapsing ocular involvement is one of the major manifestations in ABD and can be found in 60-80% of patients, resulting in retinal vasculitis, neuropathy or panuveitis. Eye involvement can lead to blindness in the affected eye in 20-50% of cases. Steroids, cyclosporin A und azathioprin are available to treat affected eyes. Studies of new treatment modalities including interferon-alpha or TNF antibodies are under way.

Adrenal Cortex Hormones↗

Ocular manifestations of human T-cell lymphotropic virus type 1 infection.

PURPOSE OF REVIEW: Human T-cell lymphotropic virus type 1 (HTLV-1) infection is endemic in Japan, the Caribbean islands, and parts of Central Africa and South America. Known ophthalmic manifestations of HTLV-1 include malignant infiltrates in patients with adult T-cell leukemia/lymphoma, retinal degeneration, neuroophthalmic disorders, and keratoconjunctivitis sicca in patients with HTLV-1-associated myelopathy/tropical spastic paraparesis, and HTLV-1-associated uveitis. This report reviews the recent developments and ocular findings reported in patients with HTLV-1-related diseases. RECENT FINDINGS: Most of the knowledge of the ocular manifestations of HTLV-1 comes from southwestern Japan, which has the highest incidence of infection worldwide. During the past few years, however, ocular disease associated with HTLV-1 has been described in patients from other endemic areas genetically distinct and geographically distant from Japan. The most interesting of these was the recognition of corneal pathology in Brazilian and Caribbean patients with HTLV-1 that have not been described in Japanese patients. Other developments include the use of molecular techniques in the diagnostic evaluation of ocular tissues from HTLV-1 patients, and clinical studies demonstrating choroidal involvement by indocyanine green angiography in patients with HTLV-1-associated uveitis, and suggesting that retinal vasculitis unresponsive to corticosteroid therapy maybe a poor prognostic sign. SUMMARY: The spectrum of ocular disease related to HTLV-1 continues to expand. Routine evaluation of HTLV-1-infected patients is important because immune-mediated or neoplastic ocular involvement may occur during the disease course. Genetic and environmental factors may play a role in the ocular manifestations of HTLV-1 in different populations.

Brazil↗

Pathologic observations made by retinal biopsy.

The authors report four cases in which retinal biopsy findings yielded unexpected or previously unreported diagnoses in patients with inflammatory retinitis. The tissue diagnoses included Wegener's retinal vasculitis in an immunosuppressed patient with a clinical diagnosis of cytomegalovirus retinitis, a novel viral form in the retina of a patient with cytomegalovirus retinitis, a case of acute retinal necrosis due to cytomegalovirus infection in an immunologically normal adult, and a case of ganciclovir-resistant herpes family viral retinitis. These cases illustrate the use of retinal biopsy in obtaining tissue for diagnosis and guiding treatment in selected cases of retinitis.

Acquired Immunodeficiency Syndrome↗

A randomized, masked, cross-over trial of lisinopril for inflammatory macular edema.

PURPOSE: To analyze the effect of angiotensin-converting enzyme (ACE) inhibitor lisinopril on inflammatory cystoid macular edema and visual acuity. DESIGN: Randomized, double-blind, placebo-controlled cross-over trial. METHODS: setting: Outpatient clinic of the Department of Ophthalmology at the University Medical Center of Utrecht. patients: Forty patients with inflammatory cystoid macular edema were included. intervention: Each patient received lisinopril (10 mg daily) or placebo for three months. After two months of a lisinopril/placebo free wash-out period, the groups received the reverse study medication for three months. Fluorescein angiography was performed to evaluate the retina. main outcome measures: Cystoid macular edema, best-corrected visual acuity and contrast sensitivity. RESULTS: Lisinopril had no effect on cystoid macular edema, visual acuity, papillary leakage, retinal vasculitis, and choroidal leakage. In a subgroup analysis, we observed a decrease in blood pressure (lisinopril, 14 of 36 patients; placebo, 5 of 36 patients; P = .02) and a decrease in morning urinary albumin excretion (lisinopril, 23 of 35 patients; placebo 10 of 34 patients, P = .003) was observed. CONCLUSIONS: Although lisinopril had no effect on inflammatory cystoid macular edema and visual acuity, we found a positive effect on the vascular system.

Adult↗

The effect of non-steroidal anti-inflammatory drugs on inflammatory cystoid macular edema.

PURPOSE: To assess the role of systemic non-steroidal anti-inflammatory drugs (NSAIDs) in the treatment of inflammatory cystoid macular edema (iCME). DESIGN: Pilot study. METHODS: We included 66 patients with iCME and treated them with naproxen (2 x 250 mg daily; n = 28) or rofecoxib (1 x 25 mg daily; n = 38). After 4 months of therapy, visual acuity and inflammation activity were measured. We evaluated the grade of CME, retinal vasculitis, and papillary leakage with fluorescein angiography. RESULTS: No clear effect of NSAIDs on iCME, visual acuity, and intraocular inflammation was observed. A beneficial effect was noted in less than 8% of the affected eyes. The drop-out rate after 4 months of treatment was 52%, because of adverse effects or inefficacy of the treatment. Improvement of visual acuity was slightly better in patients who were treated with naproxen compared with rofecoxib (P < or = .05). CONCLUSION: Systemic NSAIDs have a limited role (if any) in the treatment of iCME.

Adolescent↗

Chemokine gene polymorphisms associate with gender in patients with uveitis.

Uveitis is an inflammatory condition of ocular tissue characterized by leukocyte infiltration, tissue damage, and decreased visual acuity. Chemokines have been implicated in the pathogenesis of uveitis. Polymorphisms in the genes encoding chemokines have been described as affecting chemokine production or function. We analyzed the frequency of single-nucleotide polymorphisms (SNPs) in genes encoding CCL2 (-2518 and -2076) and CCL5 (-403 and -28) in patients with Behçet's disease (BD), a systemic form of uveitis, and patients with retinal vasculitis (RV), an organ-specific form of disease. We report that there was no association between any SNP and disease. However, when segregated on the basis of gender the CCR5 -403 AA genotype was only found in male patients with BD. Similarly, CCL2 genotypes 1/2 were predominant in males, while genotype 4 was significantly associated with disease in female patients with BD. Differences in disease symptoms and severity between males and females have been described in BD and gender-specific genetic differences in chemokine gene function may be involved.

Behcet Syndrome↗

Pars planitis in the Mexican Mestizo population: ocular findings, treatment, and visual outcome.

PURPOSE: To describe the clinical manifestations of classic pars planitis (CPP) in Mexican patients. We report here the most frequent complications, medical and surgical treatment, and visual prognosis. MATERIAL AND METHODS: A retrospective, descriptive case series examined the clinical features, complications, and treatment (medical and surgical) of CPP patients seen at the Inflammatory Eye Disease Clinic from January 1990 to September 1999. RESULTS: One hundred and sixty patients met inclusion criteria for the study. Mean age at presentation was 10 years and males were more frequently affected. Both eyes were affected in 84.4% of the cases. The most frequent complaint was decreased visual acuity. Initial visual acuity (VA) ranged from no light perception to 20/20 (mean 20/50), and mean final VA was 20/30. The most frequent clinical manifestations were vitritis (99.7%), snowballs (99.3%), retinal vasculitis (89.2%), and snowbanks (63.1%). The most common complications were cystoid macular edema (63.4%) and cataract (47.5%). Periocular corticosteroids were used in 97.5% of cases, systemic corticosteroids in 68.1%, and other immunosuppressive drugs in 21.3%. CONCLUSIONS: CPP in the Mexican population is more frequent in males and usually presents in patients less than 14 years of age. It is typically bilateral, and the most common symptom is decreased visual acuity. The most important clinical findings are located in the vitreous and retina. Cataract and cystoid macular edema are the most frequent complications. Treatment comprises periocular and systemic corticosteroids or other immunosuppressive drugs.

Administration, Topical↗

[Comparative evaluation of the effectiveness of corticosteroids and cytostatics in treating retinal angiitis in Behcet's disease].

Posterior uveitis and retinal vasculitis (RV) are the main causes of severe loss of vision or blindness in patients with Behçet's disease (BD). Treatment of RV in BD is a difficult problem, because the agents are effective in posterior uveitis and ineffective in RV. We observed 26 RV BD patients (20 men and 6 women aged 18-46 years, mean age 30.8 +/- 6.5 years), who were treated with corticosteroids and cytostatic drugs. Corticosteroids were used by pulse therapy, orally, and locally (periocular injections) during the active period. The results of pulse-therapy or oral corticosteroids were better than of periocular injections (92.8, 90.9, and 45.5%, respectively, p < 0.009). The velocity of reduction of inflammation and the degree of visual acuity improvement were better in the patients who received steroid pulse-therapy (12.7 +/- 2.4 and 0.19 +/- 0.21 days, respectively) in comparison with those who received oral steroids (17.7 +/- 1.8 days, p < 0.000, and 0.08 +/- 0.07, p = 0.0121). A combination of cyclophosphamide (200 mg/week intravenously) with prednisolone (10-15 mg/day) and cyclosporin (3.5 mg/kg/day) with prednisolone (10-15 mg/day) were effective for long control of RV BD patients.

Adrenal Cortex Hormones↗

Immunoglobulin and complement studies in children with Schönlein-Henoch Syndrome and other vasculitic diseases.

In 35 children with Schönlein-Henoch Syndrome (SHS) serum IgG, IgM, and IgA concentrations were increased in 15%, 21%, and 44% of cases, respectively. Seven children with other vasculitic syndromes (VS) had normal serum Ig concentrations. Serum concentration of IgG subclass IgG1 was increased in 72% of children with SHS and 57% with VS. In SHS this was related to the presence of arthritis, but inversely related to nephritic symptoms. Only a few children had IgG subclasses IgG2, IgG3, or IgG4 concentrations outside the normal ranges. Platelet associated Ig (PAIg) was found in 75% of children with SHS or VS. In SHS the identification of increased amounts of PAIgG was related to the presence of nephritis. The serum concentration of properdin, a component of the alternative complement system, was reduced in 21% of children with SHS. This was related to the presence of abdominal symptoms or nephritis. No cases of retinal vasculitis was observed, but 4 of 22 children with SHS had punctuate retinal haemorrhages. SHS and VS may be clinical variations of the same syndrome. The immunological aspects indicate a close relationship with autoimmune diseases.

Adolescent↗

Late ocular manifestations in neonatal herpes simplex infection.

Five patients who had had neonatal herpes simplex virus infection were re-examined at ages that varied from 6 to 9 years. In all of them the diagnosis had been supported by virus isolation and antibody titration, and herpes virus type 2, the genital strain, had been the causative agent. Only 1 of the patients was mentally retarded. Three of the patients had manifest squint, and only 1 patient had normal visual acuity in both eyes. The fundus changes varied from atrophy of the retinal pigment epithelium in 3 patients to atrophy of the optic disc in 2 eyes and extreme tortuosity of the retinal arterioles and veins in 1 patient. One patient developed bilateral retinitis with exudation into the vitreous at the age of 5 years. Fluorescein angiography of this patient showed peripheral vascular closure and preretinal neovascularisation, with leakage in both eyes. This may have been due to chronic retinal vasculitis; haemorrhagic infarction may have induced the damage to retinal tissues. Laser photocogulation resulted in slight improvement of the visual acuity, clearing of the vitreous, and decrease of the fluorescein leakage.

Child↗

Retinal fibrovascular proliferation associated with Nocardia subretinal abscess.

PURPOSE: To report the development of extensive fibrovascular proliferation in association with Nocardia subretinal abscess. METHOD: Case report. RESULTS: Extensive retinal neovascularization with tractional retinal detachment developed soon after ocular involvement in a 61-year-old patient with systemic nocardiosis. Fundus fluorescein angiography showed extensive area of capillary nonperfusion and severe leakage from the neovascular complex. The Nocardia subretinal abscess responded to systemic antibiotics, and the retinal neovascularization and tractional retinal detachment stabilized after 3 months. CONCLUSIONS: Retinal ischemia and severe retinal neovascularization may complicate intraocular nocardiosis. The authors propose secondary retinal vasculitis as a contributing factor towards the development of retinal ischemia in this setting.

Abscess↗

Virus RNA persists within the retina in coronavirus-induced retinopathy.

The murine coronavirus, mouse hepatitis virus (MHV), JHM strain, induces a biphasic retinal disease in adult BALB/c mice. In the early phase, Day 1 to Day 7, a retinal vasculitis is noted which is associated with the presence of viral proteins and infectious virus. In the late phase, Day 10 to Day 140, a retinal degeneration is associated with the absence of viral proteins, infectious virus, and inflammatory cells. The purpose of this study was to determine if viral RNA persists within the retina during the retinal degenerative phase of the disease. BALB/c mice were inoculated by the intravitreal route with 10(4.0) TCID50/5 microliters of virus. The presence of viral RNA was detected by in situ hybridization with a viral cDNA probe and viral proteins were identified by immunocytochemical staining. During the acute phase of the infection, viral RNA was found in the retina, RPE, ciliary body epithelium, and the iris epithelium. During the late phase of the infection, viral RNA was almost exclusively found within the retina and RPE and not in the anterior segment of the eye. Within the retina, viral RNA was detected in the ganglion cell layer, the inner retina, the outer retina, and the RPE cell. Immunocytochemical staining identified viral protein within the retina only from Day 1 to Day 8. This ocular disease was also associated with a persistent systemic infection. Both viral RNA and viral proteins were identified within the liver during the first 8 days. However, only viral RNA was detected in the liver from Day 8 to Day 60. These studies demonstrate that MHV established an acute infection (Day 1-8) where infectious virus and viral proteins were identified. This was followed by a persistent infection within the retina and liver where only viral RNA were detected by in situ hybridization.

Animals↗

Retinopathy in systemic lupus erythematosus.

A 32-year-old black woman with systemic lupus erythematosus went rapidly blind due to severe bilateral retinal vasculitis. The mechanism appeared to be total arrest of the retinal circulation by thrombosis of most of the retinal vasculature including major arterioles.

Adult↗

Bilateral multifocal retinal arteriolar sheathing as the only ocular finding in hereditary amyloidosis.

PURPOSE: To report the early ocular findings in hereditary amyloidosis. METHODS: Case report of a visually asymptomatic, 52-year-old Mexican man with systemic manifestations of amyloidosis (peripheral neuropathy, restrictive cardiomyopathy), with a positive family history, and with amyloid on an endomyocardial biopsy specimen, who had bilateral multifocal sheathing or cuffing of the peripheral retinal arterioles. RESULT: An extensive evaluation for retinal vasculitis was discontinued when hereditary amyloidosis was diagnosed. CONCLUSION: Focal retinal periarteriolar plaques of amyloid may be the earliest and only ocular finding of hereditary amyloidosis.

Amyloid↗

Retinal vascular nonperfusion and retinal neovascularization as a consequence of cytomegalovirus retinitis and cryptococcal choroiditis.

BACKGROUND: Cytomegalovirus retinitis and cryptococcal choroiditis are opportunistic infections in patients with acquired immune deficiency syndrome. These infections are associated with a retinal vasculitis and vascular attenuation. METHODS: We present a case of retinal vascular nonperfusion and retinal neovascularization in a patient with acquired immune deficiency syndrome. RESULTS: Retinal vascular nonperfusion and retinal neovascularization were confirmed by fluorescein angiography. CONCLUSIONS: This is the first reported case of retinal neovascularization in a patient with acquired immune deficiency syndrome, ocular cytomegalovirus retinitis, and cryptococcal infection.

AIDS-Related Opportunistic Infections↗

[Characteristics of retinal vascular involvement in systemic lupus erythematosus].

Retinal vascular abnormalities were studied in 194 patients with systemic lupus erythematosus (SLE). All patients fulfilled the American Rheumatism Association criteria for SLE. The mean age of patients was 31.9 +/- 9.7 years (17-63 years), women falling ill 5 times more often than men. Retinal vascular abnormalities were found in 67 (34.5%) patients and were as follows: retinal angiopathy (80.6%), cotton-wool spots (10.4%), occlusion of central vein or its branches (3%), occlusion of a retinal artery branch (4.5%), and retinal vasculitis with extensive peripheral capillary nonperfusion and neovascularization (3%). In general, retinal vascular occlusions were found in 6.7% of all SLE patients and in 19.4% of SLE patients with retinal vascular changes. Retinal vascular occlusions in SLE patients were associated with the antiphospholipid syndrome. Retinopathy did not depend on systemic hypertension or duration of SLE but correlated with disease activity. Small retinal vessels were involved more often than large vessels (p < 0.0395) and arteries more often than veins (p = 0.0338). Visual outcomes were better in patients with cotton-wool spots than in those with severe retinal vaso-occlusive disease (0.92 +/- 0.09 and 0.15 +/- 0.13, respectively, p < 0.0000).

Adolescent↗

Anticardiolipin antibodies and retinal vascular complications in Behçet's disease.

BACKGROUND AND OBJECTIVE: Intravascular thrombosis is a well-recognized feature of Behçet's disease (BD). In this study, the existence of a connection between serum anticardiolipin (aCL) antibodies and retinal vascular complications in BD is investigated. PATIENTS AND METHODS: Serum aCL antibody levels were quantified in 31 patients with BD, all of whom fulfilled the major criteria of the disease. Patients were evaluated in terms of retinal vascular complications. Serum aCL antibody levels were determined through the ELISA method. Statistical analysis was performed with the chi-squared test and the Fisher exact test. RESULTS: Of 12 patients with high aCL antibody levels, retinal vascular thrombosis was detected in 5 and retinal vasculitis was present in 8. However, there was no statistically significant relation between these complications and serum aCL antibody elevation (P > .05). CONCLUSION: Based on this series of 31 patients with BD, there does not seem to be a significant correlation between retinal vascular complications and serum aCL antibody levels.

Adolescent↗

Rift Valley fever ocular manifestations: observations during the 1977 epidemic in Egypt.

Ocular manifestations resulting from Rift Valley fever (RVF) virus infection were studied during an extensive RVF epidemic in Egypt during 1977. Colour photography and fluorescein angiography of 7 serologically diagnosed patients showed the commonest manifestations to be macular, paramacular, and/or extramacular retinal lesions, often occurring bilaterally. Haemorrhage and oedema were frequently associated with the lesions, and vasculitis, retinitis, and vascular occlusion were also observed. Patients were monitored during a 6-month convalescence, and, though resorption of the lesions occurred, approximately half the patients experienced permanent loss of visual acuity. Ocular disease was one form of the clinical spectrum of RVF; acute febrile, encephalitic, and fatal haemorrhagic RVF illnesses were also observed during the epidemic.

Adult↗