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Atypical presentations of ocular toxoplasmosis.

The diagnosis of ocular toxoplasmosis is based most often on the presence of characteristic clinical findings, which include focal retinochoroiditis, an adjacent or nearby retinochoroidal scar, and moderate to severe vitreous inflammation. However, a variety of less common, "atypical" presentations may be unfamiliar to clinicians, delaying both diagnosis and treatment. Patients who are immunocompromised or elderly may, for example, present with large, multiple and/or bilateral lesions. Other unusual manifestations include punctate outer retinal toxoplasmosis, retinal vasculitis, retinal vascular occlusions, rhegmatogenous and serous retinal detachments, a unilateral pigmentary retinopathy mimicking retinitis pigmentosa, neuroretinitis and other forms of optic neuropathy, and scleritis. Although in the past most cases of ocular toxoplasmosis were considered to result from reactivation of a congenital infection, it is now believed that postnatally acquired infection accounts for many cases of this disease. With appropriate use of antiparasitic therapy, the visual prognosis for patients with both typical and atypical forms of ocular toxoplasmosis may be good.

Choroiditis↗

[Ocular complications in systemic lupus erythematosus--choroidal and retinal pigment epithelial changes].

PURPOSE: Cotton wool patch and retinal hemorrhage are common ocular findings in systemic lupus erythematosus(SLE). Immunosuppressive treatment with corticosteroids may modify choroidal and retinal findings. We examined ocular complications, mainly choroidal and retinal pigment epithelium (RPE) changes in patients with SLE. MATERIALS AND METHODS: Fifty SLE cases[3 males, 47 females, 41 +/- 15 (mean +/- standard deviation) year-old] were examined and treated in the departments of Internal Medicine and Ophthalmology of Osaka University Medical Hospital. RESULTS: Complications included cataract in 16 cases, glaucoma or ocular hypertension in 9 cases, and choroidal and retinal changes in 15 cases. Occlusive retinal vasculitis, retinal pigment epithelial dysfunction, such as pigment epithelial detachment, choroidal neovascularization and multiple posterior pigment epitheliopathy, in 6 cases, and branch retinal vein occlusion in 2 cases were detected as fundus complications. Average time period from the first detection of SLE was 13 years in patients with RPE changes. CONCLUSION: Long term corticosteroid therapy is probably responsible for the RPE complications.

Adult↗

An epizootic of blindness and encephalitis associated with a herpesvirus indistinguishable from equine herpesvirus I in a herd of alpacas and llamas.

Blindness characterized by dilated unresponsive pupils and funduscopic evidence of varying degrees of vitritis, retinal vasculitis, retinitis, chorioretinitis, and optic neuritis developed in 21 alpacas and 1 llama within a 30-day period. The animals were part of a group of approximately 100 animals imported from Chile one year earlier. The animals had spent 6 months in quarantine and then, for the 6 months preceding the epizootic, were housed at an exotic animal import-export farm, where the disease developed. Four of the affected animals also had signs of neurologic dysfunction. A herpesvirus indistinguishable from equine herpesvirus I was isolated from 4 of the affected animals, and antibody titers diagnostic for equine herpesvirus I were demonstrated in the serum of all but one of the affected animals.

Animals↗

Childhood-onset uveitis in Behçet disease:a descriptive study of 36 cases.

PURPOSE: To describe the demographic and clinical features, complications, treatment, and visual results in patients with childhood-onset Behçet uveitis. DESIGN: Observational case series. METHODS: A retrospective study was made of 36 consecutive patients with Behçet disease seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, between January 1975 and January 2002. Inclusion criteria were fulfillment of the classification criteria of the International Study Group for Behçet Disease and onset of uveitis at 16 years of age or younger. The medical records of 36 patients with childhood-onset Behçet uveitis were reviewed. The main outcome measures were sex, age at onset of uveitis, the initial symptom of Behçet disease, clinical ocular features, ocular complications, systemic treatment, complications of treatment, and final visual acuity. RESULTS: Twenty-five patients were male, 11 were female. Mean age at onset of uveitis was 13.6 years. The initial symptom was oral ulcer in 63.8% of the patients. The majority of patients (83.3%) had bilateral involvement. Panuveitis was the most common form (86.2%). Retinal vasculitis and retinitis were the most common ocular findings seen in 83.3% and 68.2% of the involved eyes, respectively. Cataract, maculopathy, and optic atrophy were the most common complications seen in 46.9%, 45.4%, and 39.4% of the involved eyes, respectively. Immunosuppressive therapy was administered to 75% of the patients. Response to treatment was variable. The most common complications of systemic treatment were associated with corticosteroid therapy. Final visual acuity was worse than 0.1 in 22.7% of the involved eyes. CONCLUSIONS: Childhood-onset Behçet uveitis was more common among males. Bilateral panuveitis with retinal vasculitis and retinitis was the most common form of ocular involvement, similar to the adult patient. The treatment is challenging, as the use oral corticosteroids is associated with significant complications and the response to conventional immunosuppressive therapy is variable.

Adolescent↗

Acute retinal necrosis.

Acute retinal necrosis (ARN) is an uncommon intraocular inflammatory syndrome characterized by severe and diffuse uveitis, retinal vasculitis, and retinal necrosis. It is typically described to occur in immunocompetent patients, but can also be found in immunocompromised subjects. Varicella-zoster virus (VZV), herpes simplex virus (HSV 1 and 2), cytomegalovirus (CMV), and Epstein-Barr virus (EBV) have been implicated in the etiology of ARN. The characteristic features of the disease include iridocyclitis, vitritis, retinal vasculitis, and retinal necrosis. Bilateral involvement occurs in two-thirds of the patients, frequently in the first six weeks, but sometimes months to years later. Retinal detachment occurs in 75% of the cases. The diagnosis of ARN is usually based in clinical features. The use of polymerase chain reaction (PCR) in aqueous humor samples is useful to identify the etiology of the disease. The treatment of ARN includes intravenous acyclovir, corticosteroids and aspirin. To prevent fellow eye involvement, intravenous acyclovir is followed by oral acyclovir for 14 weeks. Alternatives to acyclovir include ganciclovir, foscarnet, famcyclovir, brivudine, and valgancyclovir.

Antiviral Agents↗

Crohn's disease and retinal vascular disease.

Patients with Crohn's disease may manifest extraintestinal findings including ocular involvement. We treated two patients with Crohn's disease who manifested retinal vascular disease that may have been related to the immune nature of the underlying disease or possibly to changes in the vasculature or coagulation system. One patient had a central retinal vein occlusion and the other had retinal vasculitis involving retinal arteries and veins with an apparent branch retinal artery occlusion. Both patients were in excellent health except for Crohn's disease, which was confirmed by a biopsy specimen. In one patient, the diagnosis of retinal vasculitis preceded the clinical diagnosis of Crohn's disease, whereas for the second patient the symptomatology of the intestinal disease preceded the ocular manifestations. We suggest that inflammatory bowel disease should be considered in the differential diagnosis of retinal vascular occlusive disease, especially in a young patient.

Adult↗

Ocular vasculitis: a multidisciplinary approach.

PURPOSE OF REVIEW: The ophthalmologist has direct visual access to inflamed vessels when examining the retina, and "vasculitis" in ophthalmology has so far mainly referred to retinal vasculitis. In the past few years the means to explore vasculitis in the ocular sphere have improved. Indocyanine green angiography now enables the analysis of choroidal inflammatory vasculopathy as well as vasculitis of the sclera (scleritis) and episclera (episcleritis) in addition to retinal vasculitis. Because vasculitis detected by the ophthalmologist can be the presenting sign of a systemic disease and has to be approached in a multidisciplinary fashion, the emerging term "ocular vasculitis," instead of retinal vasculitis, should be used in the future. The term covers vasculitis affecting all structures of the eye and the periocular tissues as detailed in this article. The ocular findings have to be integrated within the established and accepted classification of systemic vasculitis, which is divided into primary vasculitides, where the vessel itself is the target of the inflammatory reaction, and secondary vasculitides, caused by other inflammatory processes. This review will deal with recently published articles on ocular vasculitis, including its clinical aspects, its link with systemic diseases, and its investigation and management. The discussion will be conducted within the framework of the new classification put forward here. RECENT FINDINGS: Novel imaging techniques such as indocyanine green angiography have made it possible to explore inflammation of choroidal vessels and of scleral vasculitis in addition to retinal vasculitis, contributing to the global concept of ocular vasculitis. It has been shown, in particular, that the choriocapillaris, a vascular structure adjacent to the retina, can be the site of a primary inflammatory vasculopathy unrecognized so far. Most of the recent articles cited, however, deal not so much with new findings but with the integration of ocular pathologic changes into the systemic diseases they are part of. New knowledge about disease mechanisms and novel therapeutic modalities with biologic agents cited in this review are coming from other fields but have contributed to progress in the management of ocular vasculitis. SUMMARY: New investigational techniques of vasculitis in ocular structures other than the retina have contributed to the development of the global concept of ocular vasculitis. This review shows the importance of promoting a comprehensive and global classification of ocular vasculitis compatible with the concepts accepted for systemic vasculitis to contribute to its multidisciplinary approach.

Diagnostic Techniques, Ophthalmological↗

[Retinal vascularization in sarcoidosis].

OBJECTIVES: To describe the characteristics of retinal vasculitis related to sarcoidosis, and to study the radio-clinical findings of sarcoidosis in case of retinal vasculitis. METHODS: We performed a retrospective study on 33 cases of retinal vasculitis associated with sarcoidosis. Our patients had a complete ophthalmological examination and a systematic fluorescein angiography was performed. Recorded data included clinical, biological and radiological evaluation of sarcoidosis. RESULTS: Retinal vasculitis were and remained asymptomatic in almost a third of the patients (n = 10). In 11 patients (33%) a decreased visual acuity of more than 3 lines was observed. Periphlebitis was observed in all cases, and 75.7% were of non ischemic forms. Thirty patients out of 33 (90.9%) were treated with systemic steroids in whom 17 were treated for ophthalmological reasons. The presence of anterior or posterior uveitis, more than one attack of vasculitis, and significant hyalitis were statistically associated with visual acuity decrease. Ninety percent of patients regained their initial visual acuity, only three patients at the end of the medical follow-up remained with a visual acuity below or equal to 2/10. Thirty one patients (91%) presented with mediastinal and pulmonary signs concordant with sarcoidosis on chest x-rays. Extrapulmonary localisations were common in case of retinal vasculitis (91% of patients). CONCLUSION: Retinal vasculitis associated with sarcoidosis are often asymptomatic. In most cases, there are bilateral, non ischaemic periphlebitis. They seem to have a relatively better prognosis than in other etiologies of vasculitis and they are part of an evolutive multisystemic sarcoidosis. Most of the time treatment with systemic steroids was indicated for ophthalmological or extra ophthalmological reasons.

Administration, Oral↗

Bilateral panuveitis in a child with hypohidrotic ectodermal dysplasia.

PURPOSE: To report a case of bilateral panuveitis in a patient with hypohidrotic ectodermal dysplasia. DESIGN: Interventional case report. METHODS: A 6-year-old African-American boy with hypohidrotic ectodermal dysplasia presented with pain, photophobia, and decreased vision in both eyes. RESULTS: Findings included severe bilateral panuveitis with optic disk edema, macular epiretinal membrane, peripheral retinal vasculitis, and retinitis. All other known causes of panuveitis were explored and ruled out. CONCLUSIONS: The abnormal development of tissues of ectodermal origin evident in hypohidrotic ectodermal dysplasia may include a predisposition to panuveitis.

Child↗

Autoimmunity and the outer retina.

Structurally and therefore antigenically the retina is a complex tissue. Since it develops as an extension from the neural tube it shares with the brain several cell membranes and cytoplasm associated antigens including those present in neurofilaments of the various neurones and the glial filaments of the astrocytes. The advent of monoclonal antibodies has helped to dissect, in detail, the antigenic makeup of the retina. Nervous system antigens (NS-3, 4 and 7) are generously represented in the retina. At least in the chick eye there seems to be a concentration gradient of retinal antigens along a dorsoventral axis which is believed to provide means by which neurones of developing retinal signal and receive the positional information necessary for the formation of specific synapses. It now seems certain that organ-specific antigens are presented not only in the photoreceptors and the retinal pigment epithelium but also in the retinal ganglion cells and the astrocytes. Photoreceptor outer-segment contains soluble antigens which when injected in rats, rabbits, guinea-pigs or monkeys produce varying degrees of intraocular inflammation leading to uveitis, retinal detachment, photoreceptor degeneration and occasionally retinal vasculitis. Both cell-mediated and humoral immunity to photoreceptor antigen has been demonstrated in various types of uveitis (including toxoplasmosis and sarcoidosis), pars planitis, vitriitis, Behçets disease, sympathetic ophthalmitis, Vogt-Koyanagi-Harada syndrome, birdshot retinopathy, retinitis pigmentosa and retinal vasculitis. Retinal autoimmunity is also found in retinal detachment and diabetic retinopathy, particularly after Argon laser photocoagulation. Antibodies to retinal antigens are also found in patients with systemic lupus erythematosus and other systemic immune disorders without ocular involvement. The precise pathogenetic role of retinal autoimmunity in eye disease is therefore uncertain. It may simply represent an epiphenomenon which develops afer retinal damage due to physical, micro-organismal or immunological insult. Alternatively it is possible that although autoimmunity does not initiate ocular inflammation it perpetuates and maintains the inflammatory state and produces further damage to ocular tissues.

Animals↗

[The morphologic structure of vitreous hemorrhage and posterior vitreous detachment].

OBJECTIVE: To investigate the morphologic structures of vitreous hemorrhage that may be beneficial to its treatment. METHODS: The clinical data of 74 cases (79 eyes) with vitreous hemorrhage resulting from proliferative diabetic retinopathy, retinal vasculitis and retinal vein occlusion in December 1995 to January 2000 and having undergone vitrectomy were studied retrospectively, which focused on the morphologic characteristics of vitreous body by B-scan, surgical microscopy and surgical manipulation, to evaluate the relation of retina and vitreous body in order to discern the relationships among the retina, neovascular fibromembrane and the posterior hyaloid surface. RESULTS: Posterior vitreous detachment (PVD) in various degrees was observed in all the cases. The morphologic character was classified as the complete and partial vitreous detachment. The latter one can be subdivided into "V", "L" types and posterior vitreoschisis that was not rarely seen in the proliferative phase of ischemic retinopathy. CONCLUSIONS: To evaluate the characteristics of posterior vitreous detachment and vitreoschisis may provide valuable information for vitreoretinal surgery, elevate the rate of success and improve the prognosis of the surgery.

Adult↗

[Laser coagulation and cryocoagulation in the treatment of isolated retinal angiitis].

Isolated retinal vasculitis (IRV) is a form of retinal vasculitis without other inflammations of the eye or systemic vasculitis. Occlusion of retinal vessels without subsequent retinal ischemia and neovascularization is one of the main problems in the management of such patients. Laser photocoagulation seems to be a method to control this problem. Argon laser photocoagulation was used in the treatment of 34 IRV patients (56 eyes) (29 men and 15 women aged 25-41 years, mean age 32.9 +/- 5.2 years). A total of 168 sessions were carried out. Regression of neovascularization was achieved in 29 of 44 eyes (79.5%). Additional cryotherapy had to be performed in 15 eyes, good results were attained in 9 eyes (60%). Patients treated with prednisolone in a daily dose of 10-20 mg during laser coagulation had IRV recurrences more rarely than those treated with lower doses (5 mg) (0-6.7% vs. 50%, p < 0.0013), those treated with metipred-depot for 7-14 days (0-6.7% vs. 53.8-66.6%, p < 0.0023), or untreated ones (0-6.7% vs. 73.9%, p < 0.0000).

Adult↗

[The clinical features of 9 cases of intraocular malignant lymphoma of oculocerebral origin].

We reviewed a series of 13 eyes in 9 cases of intraocular lymphoma with or without intracranial involvement during the past 15 years. The cases were characterized by moderate or no inflammation in the anterior ocular segment and by the presence of vitreous opacity and fundus lesions. Contrary to the accepted view that this disease simulates uveitis with various manifestations, the fundus lesions in the present series showed disseminated or tumorous subretinal lesions suggestive of proliferation of tumor cells. Only a few cases showed retinal vasculitis or retinal exudates. Electrooculograms showed findings suggestive of widespread impairment of the retinal pigment epithelium. Because of poor prognosis and sensitivity to radiation, we advocate early diagnosis and treatment for this disease.

Adult↗

Anticardiolipin antibodies in uveitis.

Anticardiolipin antibodies (ACA) have been described in association with thrombosis and several vascular diseases and may be involved in retinal vasculitis. To investigate this question we tested sera from 86 patients with posterior uveitis and 100 healthy controls for the presence of anticardiolipin antibodies. The antibody was present in 15 patients: in 7 of the patients with acute retinal necrosis (n = 24), in 4 with idiopathic retinal vasculitis (n = 10), 3 with syphilis (n = 6) and in one SLE patient with retinal vasculitis. Ten patients had IgA-type anticardiolipin antibodies, 6 had IgM-type and 4 had IgG-type anticardiolipin antibodies. Anticardiolipin antibodies could not be detected in sera from uveitis patients with Behçet's disease (n = 10), sarcoid (n = 9), toxoplasmosis (n = 7), multiple sclerosis (n = 3) or Birdshot chorioretinopathy (n = 10). Ten of the healthy controls had cardiolipin antibodies of the IgM class. The exact role of these auto-antibodies in the pathogenesis of retinal vasculitis remains to be resolved.

Antibodies, Anticardiolipin↗

Retinal manifestations of ocular lymphoma (reticulum cell sarcoma).

BACKGROUND: Diagnosis and treatment of ocular large cell lymphoma may lessen visual loss and prolong life. Although reports in the literature have described retinal infiltrates in eyes with large cell lymphoma, they have focused on the more prominent vitreous and subretinal pigment epithelial findings. Eyes with retinal infiltrates and hemorrhagic retinal necrosis are usually believed to harbor a microbial infection. The authors describe 5 patients, aged 57 to 85 years, with ocular lymphoma in whom the most prominent initial findings were in the retina. METHOD: Patients presented with findings suggestive of an infectious retinal necrosis. When the initial therapy failed, investigators performed a vitreous biopsy. Two patients had a concomitant retinal biopsy. Radiation therapy was given to four patients. RESULTS: All five patients had vitreous cells. Three patients had prominent perivascular exudate. Four patients had grayish-white retinal infiltrates, and three patients had associated retinal hemorrhage. Three patients had subretinal small white spots. An edematous thickened retina developed in three patients, and hemorrhagic retinal necrosis developed in three patients. The initial diagnosis was believed to be acute retinal necrosis (ARN) in three patients, toxoplasmosis in one patient, and frosted branch angiitis in one patient. Vitreous biopsy was positive in two patients but negative in three patients. In two of these three patients, the diagnosis was established by retinal biopsy. CONCLUSION: Ocular lymphoma should be considered in the differential diagnosis of retinal vasculitis or necrotizing retinitis in a middle-aged or older patient. Retinal biopsy may be helpful in establishing the diagnosis.

Aged↗

Immunocytochemical analysis of blood lymphocytes in uveitis.

We studied the surface expression of activation markers IL2-R, HLA-DR and CD45-RO on peripheral T-lymphocytes in two groups of patients (n = 26) with idiopathic uveoretinitis, compared with controls. Thirteen patients were analysed by alkaline phosphatase anti-alkaline phosphatase (APAAP) immunocytochemistry, which demonstrated a significant rise in expression of HLA-DR and IL2-R surface markers. Flow cytometric analysis was performed on a further 13 patients, which confirmed a significant rise in IL2-R expression in uveitis patients. Within this group systemic activation was confined to patients with idiopathic retinal vasculitis. Dual flow cytometry confirmed a CD4+,IL2--R+ T--lymphocyte phenotype. A further 4 patients with retinal vasculitis who had been treated with cyclosporin A demonstrated a 32% reduction in IL2-R expression over a 3-month period. Analysis of CD45-RO and CD5+ cells was found to be uninformative in this study. We have demonstrated activated peripheral lymphocytes in patients predominantly with retinal vasculitis, the significance of which is discussed.

Biomarkers↗

Genetic predisposition to coronavirus-induced retinal disease.

PURPOSE: Retinal inflammatory and degenerative processes in humans and animals frequently are associated with genetic factors. The murine coronavirus, mouse hepatitis virus (MHV), JHM strain, induces a biphasic retinal disease in adult BALB/c mice. The genetic constitution of the host and the virus serotype can be critical factors in determining the outcome of a virus infection. The purpose of this study was to evaluate the possible role of host genetics in murine coronavirus-induced retinal disease. METHODS: JHM virus was inoculated by the intravitreal route into BALB/c, CD-1, and A/J mice. At varying times after inoculation, eye tissues were evaluated histologically. Antibody responses to the virus were evaluated by neutralization assays. RESULTS: JHM virus induces a biphasic retinal disease in BALB/c mice. In the early phase, 1 to 7 days after inoculation, retinal vasculitis is observed. The second phase, characterized by retinal degeneration in the absence of inflammation, is seen by day 10 and progresses for several months. There is a similar biphasic disease process in JHM virus-infected A/J mice. However, retinal changes are less severe than those seen in BALB/c mice. Retinal tissue damage induced by JHM virus in CD-1 mice is different. Only the early phase of the disease, consisting of retinal vasculitis, was observed. These CD-1 mice do not develop the retinal degenerative disease. In fact, after day 10, the retina has a normal appearance. These differences in retinal tissue damage are seen over a wide range of infectivity of the virus inocula. Virus concentrations ranging from 10(1.4) to 10(4.4) TCID50/5 microliters were capable of inducing both inflammation and degeneration in BALB/c mice, whereas, the highest concentration of virus (10(4.4) TCID50/5 microliters) in CD-1 mice resulted in only the early inflammatory changes. CONCLUSIONS: The authors show that the genetics of the host can profoundly affect the nature of retinal tissue damage. These studies substantiate the concept that a virus can indeed trigger retinal degenerative processes in genetically susceptible hosts.

Animals↗

Central retinal artery occlusion associated with ocular Behçet's disease.

PURPOSE: To report a case of central retinal artery occlusion associated with ocular Behçet's disease (BD) and briefly discuss retinal vasculitis due to BD. CASE REPORT: A 52-year-old man, diagnosed as BD 22 years ago and followed up with ocular involvement for six years presented with sudden loss of vision. The clinical diagnosis was central retinal artery occlusion. RESULTS: No other associated systemic diseases were found and the case was classified as a complication of retinal vasculitis due to BD. CONCLUSIONS: Although the arteries are rarely affected in retinal vasculitis due to BD, it has to be considered in the differential diagnosis of retinal arterial occlusions especially in countries where the disease is prevalent. To our knowledge, this is the first reported case of ocular BD complicated with central retinal artery occlusion.

Behcet Syndrome↗