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The expanding clinical spectrum of ocular lyme borreliosis.

OBJECTIVE: To delineate the clinical manifestations of ocular Lyme borreliosis, while concentrating on new symptoms and findings and the phase of appearance of ophthalmologic disorders. DESIGN: Observational case series. PARTICIPANTS: Ten patients with Lyme borreliosis-associated ophthalmologic findings previously reported from the Helsinki University Central Hospital in addition to 10 new cases that have since been diagnosed. INTERVENTION/TESTING: The patients underwent medical and ophthalmologic evaluation. The diagnosis of Lyme borreliosis was based on medical history, clinical ocular and systemic findings, determinations of antibodies to Borrelia burgdorferi by enzyme-linked immunosorbent assay and immunoblot analysis, the detection of DNA of B. burgdorferi by polymerase chain reaction, and exclusion of other infectious and inflammatory causes. MAIN OUTCOME MEASURES: Ocular complaints, presenting ophthalmologic findings, and the stage of Lyme borreliosis were recorded. RESULTS: Four patients presented with a neuro-ophthalmologic disorder, five had external ocular inflammation, 10 patients had uveitis, and one had branch retinal vein occlusion. One patient developed episcleritis and one patient developed abducens palsy within 2 months of the infection incident. In the remaining 14 patients in whom the time of infection was traced, the ocular manifestations appeared in the late stage of Lyme borreliosis. Two patients with a neuro-ophthalmologic disorder and one with external ocular inflammation experienced severe photophobia, whereas the main reported symptom of the patients with uveitis was decreased visual acuity. Four patients with external ocular disease and one with a neuro-ophthalmologic disorder experienced severe periodic ocular or facial pain. Retinal vasculitis developed in seven patients with uveitis. CONCLUSIONS: Lyme borreliosis can cause a variety of ocular manifestations, which develop mainly in the late stage of the disease. Photophobia and severe periodic ocular pain can be characteristic symptoms of Lyme borreliosis. In the differential diagnosis of retinal vasculitis, Lyme borreliosis should be taken into account, especially in endemic areas.

Adult↗

FK506 treatment of noninfectious uveitis.

PURPOSE: We studied the clinical effects of the immunosuppressive agent FK506 in patients with noninfectious uveitis. METHODS: This study was designed as a multicenter open clinical trial in Japan. Sixteen patients with noninfectious uveitis who had visited the Uveitis Survey Clinic of the Yokohama City University Hospital were given FK506. Eight had Behçet's disease; five, Vogt-Koyanagi-Harada syndrome; one, sympathetic ophthalmia; one, retinal vasculitis; and one, sarcoidosis. In patients with Behçet's disease, ocular attack score before and after therapy was compared to judge clinical status. For the other diseases, the ocular inflammatory symptoms were observed after the initiation of FK506 treatment. All patients underwent blood and urine examinations, electrocardiography, and chest x-rays before and after FK506 treatment. RESULTS: Of the patients with Behçet's disease, five improved, one remained unchanged, one deteriorated, and the status of one could not be determined. Of the patients with Vogt-Koyanagi-Harada syndrome, four improved, and one remained unchanged. The patient with sympathetic ophthalmia improved, the patient with retinal vasculitis remained unchanged, and the status of the patient with sarcoidosis could not be determined. Major adverse effects were sensations of warmth, hypomagnesemia, renal dysfunction, glucose intolerance, nausea, vomiting, and disorders of the central nervous system. All adverse effects disappeared or improved when FK506 was stopped or when the dosage was decreased. Renal dysfunction and glucose intolerance appeared when the blood level of FK506 was high. CONCLUSIONS: FK506 was effective in patients with uveitis, but it is important to monitor the occurrence of adverse effects.

Adult↗

[Alternatives to steroid therapy in chronic uveitis].

In a search for alternative therapeutic methods other than corticosteroids and cytostatics, the effect of a dialyzable leukocyte extract (DLE), the antimetabolite 5-fluorouracil and the immunosuppressive agent cyclosporin A in corticosteroid-resistant idiopathic uveitis was studied. When DLE was administered to 26 patients who had uveitis forms with exogenous triggering (e.g., infection), as well as forms with an autoimmune background, there was a reduction in the number and duration of recurrences and a statistically proven prolongation of the inflammation-free intervals. This was particularly true in anterior and posterior uveitis and to a lesser extent in the intermediate form. No side effects were observed. 5-Fluorouracil, injected subconjunctivally, is indicated in intermediate uveitis with marked vitreous infiltration and beginning proliferation. Corneal erosion occurs relatively often. During treatment with cyclosporin A (low dose, 5 mg/kg of body weight per day), 14 of 17 patients (9 with intermediate uveitis, 6 with retinal vasculitis, 1 with sympathetic ophthalmia, 1 with panuveitis) showed improved results; in 2 cases the findings remained stationary and only 1 case had low-grade deterioration. If one takes into consideration the fact that in this patient any therapy would have failed, the results are convincing. This is particularly true of retinal vasculitis. There is no effect in cases of central hemorrhagic chorioretinopathy. So far, there have been no serious side effects.

Adrenal Cortex Hormones↗

New diagnostic approach for ocular tuberculosis by ELISA using the cord factor as antigen.

BACKGROUND/AIMS: Diagnosis of ocular tuberculosis is difficult, particularly the retinal vasculitis type, because most cases occur without concurrent active pulmonary tuberculosis. Recently, it has been reported that detection of antibodies against purified cord factor (trehalose-6,6'-dimycolate, TDM), the best studied, most antigenic, and most abundant cell wall component of tubercule bacilli, is very useful for rapid serodiagnosis of pulmonary tuberculosis. In this study, an attempt was made to evaluate whether the detection of anticord factor antibody is also useful for diagnosis of ocular tuberculosis and the necessity of antituberculous therapy for tuberculous retinochoroiditis was discussed. METHODS: Cases consisted of 15 patients with uveitis and retinal vasculitis, nine patients with presumed ocular tuberculosis, three patients with sarcoidosis, and three patients with Behçet's disease. IgG antibodies against purified cord factor prepared from Mycobacterium tuberculosis H37Rv were detected by enzyme linked immunosorbent assay. RESULTS: All cases of clinically presumed ocular tuberculosis were positive, whereas all of the cases of sarcoidosis or Behçet's disease were negative for anticord factor antibodies. When the anticord factor antibody titres were compared on the basis of the presence or absence of previous antituberculosis chemotherapy, the mean anticord factor antibody titre of the untreated group showed a tendency to be higher than in the treated group, but not significantly (p=0.07). CONCLUSIONS: The detection of anticord factor antibody may be useful to support the diagnosis of ocular tuberculosis. Additionally, a positive result for anticord factor antibody may indicate that tubercule bacilli are present in some organ(s) of the patient even in the absence of active systemic disease.

Adult↗

Thrombosis in Behçet's disease: a retrospective survey from a single UK centre.

OBJECTIVE: To contribute to knowledge of vascular occlusion in Behçet's disease (BD), prevalence and relative risk for thrombosis were ascertained retrospectively in a cohort of Caucasian patients with the disease. PATIENTS: The study included 73 BD patients (36 males, 37 females, mean age 45+/-11 yr) attending the Immunology/Rheumatology Department of Northwick Park Hospital. A group of 146 patients without BD, attending the same department, served as a control group. RESULTS: Thrombosis was more frequent in BD patients than in controls (23/73, 32% vs 7/146, 5%, P<0.001). This was accounted for by a higher prevalence of venous thrombosis in BD patients (18/73, 25% vs 4/146, 3%, P<0.001). Gender-adjusted data revealed an 11-fold risk of developing any thrombosis and a 14-fold risk of developing venous thrombosis in BD. After adjusting for differences in age at first symptoms, male BD patients showed a 6-fold higher risk of vein thrombosis. Males reported more often thrombophlebitis (13/36, 36% vs 0/0, P<0.001), folliculitis (8/36, 22% vs 1/37, 3%, P<0.01) and retinal vasculitis (13/36, 36%, vs 4/37, 11%, P=0.01) than females, in whom arthralgia prevailed (23/37, 62%, vs 12/36, 33%, P=0.01). CONCLUSION: In our population, BD confers a 14-fold risk of developing venous thrombosis. The risk is sixfold higher in male BD patients, who fare worse than females with regard to thrombophlebitis, folliculitis and retinal vasculitis.

Behcet Syndrome↗

[Ocular tuberculosis].

The frequency of tuberculous uveitis has extremely decreased in Japan. Anterior granulomatous or non-granulomatous uveitis, chorioretinitis and retinal vasculitis are common ocular manifestations, while tuberculoma, scleritis, keratitis and orbital tuberculosis are rare. The diagnosis of ocular tuberculosis is extremely difficult because ocular tuberculosis tends to be negative in chest x-ray or tuberclin skin test. To diagnose ocular tuberculosis clinically some ophthalmologists recommend subconjunctival tuberculin test or therapeutic isoniazide (INH) test. Recently, for confirmed diagnosis, polymerase chain reaction (PCR) technique has been used to detect mycobacterium in intraocular samples such as aqueous or vitreous humor. The mainstay of treatment is antituberculosis agents. Active retinal vasculitis or tuberculoma are generally responsive to corticosteroid therapy. Although ocular tuberculosis is rare, it must be considered as one of the possible causes of uveitis.

Chorioretinitis↗

Longitudinal cohort study of patients with birdshot chorioretinopathy II: color vision at baseline.

PURPOSE: To describe color vision at the baseline examination of 80 participants in a longitudinal cohort study of birdshot chorioretinopathy and to identify relationships between color vision and visual acuity, symptoms, and ophthalmic signs. DESIGN: Single center cross-sectional study. METHODS: Color vision was evaluated with the desaturated Lanthony 15-Hue test. Relationships were sought between the square root of the color confusion scores (CCS) and the following factors: best-corrected visual acuity (BCVA), symptoms, cataract, vitreous inflammatory reactions, retinal vasculitis, cystoid macular edema (CME), and birdshot lesion characteristics. RESULTS: When compared with published, age-matched normal control subjects, 49 patients (61.3%; 76 eyes [47.5%]) had abnormal CCS values. Abnormal CCS values were found in nine of 51 phakic eyes (18%) with normal BCVA (>or=1.0) and without cataract. Although most eyes did not have classifiable defects, 30 eyes (18.8%) had tritan (blue-yellow) defects (88% of eyes with classifiable defects). With the use of multivariate analyses, there were significant associations between increased CCS values and the symptoms of altered color vision (P = .005) and altered contrast sensitivity (P = .015). There was a significant, but weak, relationship between CCS values and birdshot lesion morphologic condition (P = .049), but no relationships were found with other lesion characteristics or with vitreous inflammatory reactions, retinal vasculitis, or CME. CONCLUSION: The Lanthony 15-Hue test provides an objective technique to assess complaints of altered color vision in people with birdshot chorioretinopathy. Color vision can be abnormal in eyes with normal visual acuity; therefore, this parameter may be useful as an additional measure for monitoring the disease.

Adult↗

A feline model of ocular toxoplasmosis.

UNLABELLED: PURPOSE. This study was performed to characterize the clinical, serologic, histopathologic, and immunohistochemical features of an experimental model of ocular toxoplasmosis in cats. METHODS: Seven specific pathogen-free cats were inoculated in the right carotid artery with 5 x 10(3) tachyzoites of the ME49 strain of Toxoplasma gondii. Control cats received heat-killed tachyzoites. RESULTS: Progressive, bilateral, multifocal retinal, and choroidal inflammatory foci developed in the principal cats, beginning 5 to 8 days postinoculation (PI). Lesion development peaked 3 weeks PI, and the lesions varied in size from pinpoint to 5 mm, had a predilection for the central tapetal fundus, and were more numerous ipsilateral to the side of inoculation. Resolution of the lesions 21 to 70 days PI was characterized by foci of tapetal destruction and retinal degeneration. Fluorescein angiography showed disruption of the blood-retinal barrier at the level of the retinal pigmented epithelium, and occasional retinal vasculitis and perivasculitis. Mild anterior uveitis developed in four cats 10 to 13 days PI. Aside from a slight febrile response 2 to 3 days PI, no physical abnormalities were observed. T. gondii antigens were detected intermittently in the serum of four of seven cats as early as 8 days PI. T. gondii-specific immunoglobulin M titers were present on day 7 PI and continued to increase until 28 days PI. Immunoglobulin G production was documented on day 13 PI, and titers continued to increase throughout the study. Evidence of anterior uveal antibody production (mean Goldmann-Witmer coefficient [C value], 80.7; range, 13.4 to 236.6) was present in 11 of 14 eyes on day 70 PI. On histopathologic evaluation 70 days PI, multifocal granulomatous chorioretinitis, with retinal degeneration, retinal vasculitis, and lymphocytic-plasmacytic anterior uveitis, was documented. Tissue cysts in the retina and choroid were found with mouse inoculation of tissue suspensions, immunohistochemical studies, and histopathologic examination. CONCLUSIONS: This nonfatal, noninvasive method of inducing ocular toxoplasmosis may prove to be a useful model for investigation of toxoplasmi retinochoroiditis, particularly with the recent characterization of a naturally occurring, immunosuppressive feline lentivirus with properties similar to human immunodeficiency virus.

Animals↗

Tacrolimus (FK506) in the treatment of posterior uveitis refractory to cyclosporine.

OBJECTIVE: To assess the efficacy and side effects of tacrolimus, a potent immunosuppressive macrolide antibiotic, in the treatment of sight-threatening uveitis. DESIGN: A clinical study of tacrolimus in patients who required systemic immunosuppression for control of uveitis, but were refractory to cyclosporine. PARTICIPANTS: Six patients with uveitis were treated: three had Behçet disease, one had microscopic polyangiitis, one had pars planitis, and one had idiopathic retinal vasculitis. INTERVENTION: Patients with sight-threatening uveitis refractory to cyclosporine were treated with tacrolimus. MAIN OUTCOME MEASURES: Intraocular inflammation, visual acuity (VA), neovascularization. Adverse effects of tacrolimus were documented. RESULTS: The posterior uveitis remained controlled in all patients while they were taking tacrolimus. Five of the six patients showed improvement, defined as improvement of two or more lines of Snellen acuity or a decrease in the binocular indirect ophthalmoscopy score (P < 0.05, Sign test). One patient with Behçet disease showed a marked improvement in best-corrected VA from 1/60 to 6/24. Two patients with Behçet disease showed a modest improvement in VA in the affected eye and had no disease activity in the other eye. The patient with microscopic polyangiitis was symptomatically improved, and there was no progression of the posterior uveitis. The patient with pars planitis had an improvement in VA from 6/18 to 6/9. The patient with retinal vasculitis showed partial regression of neovascularization on tacrolimus. Side effects were less troublesome than with cyclosporine. CONCLUSIONS: Tacrolimus (FK506) has a useful role as an immunosuppressive agent for the treatment of sight-threatening uveitis in patients who did not respond to cyclosporine either because of lack of therapeutic effect or unacceptable adverse effects.

Adolescent↗

Occlusive vasculitis in a patient with concomitant West Nile virus infection.

PURPOSE: To describe a patient with occlusive, retinal vasculitis and concomitant, confirmed, acute West Nile virus (WNV) infection. DESIGN: Observational case report. METHODS: Main outcome measures included comprehensive ophthalmic examination with fluorescein angiography, color photography, and serologic testing for WNV and St. Louis encephalitis (SLE) virus including plaque reduction neutralization testing (PRNT). RESULTS: A 46-year-old woman developed a sudden decrease in vision in her left eye 2 weeks after confirmed WNV infection and demonstrated multiple, small, patchy areas of retinal edema with scattered microaneurysms. Fluorescein angiography showed multiple branch artery occlusions with extensive nonperfusion. Serologic titers for WNV were positive for acute infection. Plaque reduction neutralization testing confirmed WNV infection and excluded St. Louis encephalitis virus infection. Other etiologies of occlusive vasculitis were not present. CONCLUSIONS: Occlusive, retinal vasculitis may occur in the setting of acute WNV infection.

Acute Disease↗

The acute retinal necrosis syndrome. Part 2: Histopathology and etiology.

The acute retinal necrosis syndrome is manifested by diffuse uveitis, vitritis, retinal vasculitis, and acute necrotizing retinitis (see Part 1). We studied the histopathology and electron microscopic findings of an eye enucleated from a 67-year-old man with typical acute retinal necrosis. Histology showed profound acute necrosis of the retina, retinal arteritis, and eosinophilic intranuclear inclusions in retinal cells. Electron microscopy demonstrated a herpes group virus in all layers of affected retina. The implications of these findings for antiviral and other treatments are discussed.

Acute Disease↗

Acute retinal necrosis syndrome in a child.

We recently cared for an 11-year-old child with acute retinal necrosis syndrome, an ophthalmologic condition characterized by the triad of anterior uveitis, occlusive retinal vasculitis and progressive peripheral retinal necrosis. Acute retinal necrosis syndrome occurs primarily in nonimmunocompromised adults as a result of reactivated herpes simplex or varicella-zoster virus infection. Antiviral and antiinflammatory therapy appears to reduce the incidence of vision-threatening retinal necrosis and involvement of the contralateral eye.

Anti-Inflammatory Agents↗

Regression of disc neovascularization by photocoagulation in the acute retinal necrosis syndrome.

Disc neovascularization developed in one eye of a 40-year-old white woman with the acute retinal necrosis syndrome. Krypton laser scatter photocoagulation was placed in areas of nonnecrotic and segmentally nonperfused retina that were demonstrated by fluorescein angiography. Marked reduction in disc neovascularization was noted clinically 16 days after laser treatment and was confirmed angiographically 4 weeks after treatment. Neovascularization was completely regressed at 9 weeks after treatment. The authors postulate that the retinal vasculitis and nonperfusion produced retinal ischemia which stimulated neovascular proliferation, and that photocoagulation caused regression of the neovascularization.

Adult↗

Systemic lupus erythematosus presenting earlier as retinal vaso-occlusion.

Retinal vascular lesions are the most common ophthalmologic manifestation of systemic lupus erythematosus (SLE), occurring in 3% to 29% of cases, generally late in the disease. More rare is the severe vaso-occlusive disease, often termed "retinal vasculitis", which includes central retinal artery occlusion, multifocal arteriolar occlusions, extensive capillary nonperfusion and central venous occlusion. Patients with SLE and raised serum concentrations of anticardiolipin antibodies (ACA) have a higher risk of developing occlusive ocular vascular disease. We report a case in which retinal involvement was an earlier manifestation of SLE in a patient without ACA.

Adolescent↗

Clinic analysis of Behçet disease.

PURPOSE: To analyze the clinic manifestation and prognosis of Behçet disease. METHOD: Twenty patients requiring inpatient treatment with Behçet disease were retrospectively analyzed. RESULTS: The morbidity of Behçet disease is 5.5/100 000. In the systemic damage, stomatocace and skin lesion are 95%, eye lesion and genital ulcer 50%, joint lesion 45%, gastrointestinal lesion 35%, Uveitis is the major disease in eye lesion, and followed in order by retinal vasculitis and obstruction of retinal artery. Attack age average 30.3 years old. Diagnosis age average 34.8 years old. The patients stay in hospital for 41 days on the average. Cure rate is 55%, improvement rate 40%, blinding rate of eye lesion is 36%. CONCLUSIONS: Behçet disease is a multisystem lesion disease. Stomatocace and skin lesion is the major lesion, the next in common occurrence are eye and genital lesions. Repeated attack of uveitis, complicated cataract and secondary glaucoma are the major causes of blindness.

Adolescent↗

Retinal manifestations of Mediterranean spotted fever.

The authors studied the incidence, type and systemic relationship of the retinal findings observed by ophthalmoscopy in Mediterranean spotted fever (MSF) in a consecutive series of 34 cases. It is found that retinal vasculitis either arterial and/or, more frequently, venous is present in 55.9% of the cases. Branch vein retinal thrombosis appeared as complication of such small vessel vasculitis in 1 case. Although frequent, the retinal findings were asymptomatic in all cases but the retinal vein thrombosis one, and were not significatively related to or were predicted by other systemic parameters of severity of the disease. Because of its frequency, retinal vasculitis might be considered as an important clinical sign of MSF in endemic areas.

Adolescent↗

Human T-cell lymphotropic virus type 1 infection and ocular manifestations in São Paulo, Brazil.

OBJECTIVES: Although human T-cell lymphotropic virus type 1 (HTLV-1)-associated uveitis has been well recognized in Japan, related studies in Brazil are scarce. We performed a serologic survey for HTLV-1 infection among patients with uveitis and investigated the ocular findings in HTLV-1-asymptomatic carriers. METHODS: One hundred ninety serum samples from patients with uveitis of determined (n = 137) and undetermined origins (n = 53) being examined at the Uveitis Service, University of São Paulo, São Paulo, Brazil, underwent testing using HTLV enzyme-linked immunosorbent assay and discriminatory Western blots. One hundred five asymptomatic blood donors and/or their relatives who were seropositive for HTLV-1 (carrier group) and 105 age- and sex-paired blood donors who were seronegative for HTLV-1 (control group) underwent ocular evaluation. For the statistical analysis, chi2 test was used. RESULTS: Only 1 patient with uveitis was seropositive for HTLV- 1, and she belonged to the group with uveitis of undetermined origin. Results of tear films were evaluated in 52 carriers. The prevalence of a decreased tear break-up time was significantly higher in the carrier compared with the control group (P = .02). Two carriers had keratoconjunctivitis sicca. Three of the 105 carriers exhibited mild uveitis (cells in the vitreous, retinal and choroidal infiltrates, retinal vasculitis, and bilateral pars planitis). Retinal pigmentary changes were found in both groups (no statistical difference). CONCLUSIONS: Early tear abnormalities may be present in asymptomatic carriers, and mild uveitis may be found among them. The relatively low seroprevalence of HTLV-1 in the Brazilian population made it difficult to establish the real importance of HTLV-1-associated uveitis among our patients with uveitis.

Adolescent↗