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Eales' disease.

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Humans↗

Toxoplasmosis.

Toxoplasmosis is the most common cause of posterior uveitis in immunocompetent subjects. The infection can be congenital or acquired. Ocular symptoms are variable according to the age of the subject. For instance, young children present with reduced visual acuity, strabismus, nystagmus, and leucocoria, while teenagers and adults complain of decreased vision, floaters, photophobia, pain, and hyperemia. Toxoplasmic retinochoroiditis typically affects the posterior pole, and the lesions can be solitary, multiple or satellite to a pigmented retinal scar. Active lesions present as grey-white focus of retinal necrosis with adjacent choroiditis, vasculitis, hemorrhage and vitreitis. Cicatrization occurs from the periphery towards the center, with variable pigmentary hyperplasia. Anterior uveitis is a common finding, with mutton-fat keratic precipitates, fibrine, cells and flare, iris nodules and posterior synechiae. Atypical presentations include punctate outer retinitis, neuroretinitis, papillitis, pseudo-multiple retinochoroiditis, intraocular inflammation without retinochoroiditis, unilateral pigmentary retinopathy, Fuchs'-like anterior uveitis, scleritis and multifocal or diffuse necrotizing retinitis. The laboratory diagnosis of toxoplasmosis is based on detection of antibodies and T. gondii DNA using polymerase chain reaction (PCR). Toxoplasmosis therapy includes specific medication and corticosteroids. There are several regimens, with different drug combinations. Medications include pirimetamine, sulfadiazine, clindamycin, trimethoprime-sulphamethoxazol, spiramycin, azithromycin, atovaquone, tetracycline and minocycline. The prognosis of ocular toxoplasmosis is usually good in immunocompetent individuals, as long as the central macula is not directly involved.

Animals↗

Cerebral vasculitis in Henoch-Schönlein purpura: a case report with sequential magnetic resonance imaging changes and treated with plasmapheresis alone.

Neurological complications are rare during the course of Henoch-Schönlein purpura (HSP). We report a 7-year-old girl with HSP who presented with seizures, loss of vision and disturbance of consciousness. Magnetic resonance imaging (MRI) showed high signal intensity in the gray and white matter over the left parietal and both occipital lobes, compatible with MRI findings of cerebral vasculitis. The eye fundi revealed multiple branches of retinal artery occlusion. Intravenous pulse methylprednisolone (MTP) followed by oral steroid therapy was initially administered for HSP nephritis. Cerebral vasculitis developed 10 days post-MTP treatment, with progressive worsening of consciousness. Oral steroid was discontinued and plasmapheresis was performed alone. Her level of consciousness dramatically improved after plasmapheresis. The brain MRI and eye fundi findings were consistent with her clinical improvement. To the best of our knowledge, this is the first description of MRI abnormalities and multiple retinal artery branch occlusion of cerebral vasculitis in a patient with HSP that was successfully treated by plasmapheresis alone. In conclusion, we propose that plasmapheresis may be used as a first-line therapy or rescue therapy for cerebral vasculitis in HSP.

Anti-Inflammatory Agents↗

Cytomegalovirus retinitis in Wegener's granulomatosis: case report and review of the literature.

PURPOSE: To present the first case of cytomegalovirus (CMV) retinitis in a Wegener's granulomatosis patient on systemic cyclosporine A (CsA) therapy. METHODS: A 41-year-old patient was treated with systemic CsA for c-ANCA positive Wegener's granulomatosis. Four weeks later he presented with blurred vision and a retinitis was diagnosed in the right eye. After detection of CMV DNA by polymerase chain reaction (PCR), the patient was treated with intravenous gancyclovir. Due to bone marrow toxicity, systemic antiviral therapy was discontinued and an intravitreal gancyclovir pellet was implanted. RESULTS: The retinitis lesions were scarified under this therapy and the patient's visual function was restored completely. CONCLUSIONS: Cytomegalovirus retinitis can occur under systemic monotherapy with CsA in patients with systemic vasculitis. Intraocular virus DNA detection by PCR is recommended to rule out retinal involvement of the underlying vasculitis in atypical cases and to preserve the patient's visual acuity.

Adult↗

Treatment of ocular symptoms of Behçet's disease with interferon alpha 2a: a pilot study.

AIM: To study long term effects of interferon alpha 2a (IFN alpha 2a) on panuveitis in seven patients with Behçet's disease in a prospective, open clinical trial. METHODS: Seven patients were treated with IFN alpha 2a for a mean of 23.6 months (14-37 months). They received an initial dose of IFN alpha 2a of 6 x 10(6) IU/day, followed by 3 x 10(6) IU/day after 1 month and 3 x 10(6) IU every other day after 3 months. Two patients received low dose prednisolone (between 0.2 and 0.4 mg/kg/body weight) additionally at the beginning of the therapy. Complete cessation of IFN alpha 2a was possible in three patients (observation period 22, 6, and 4 months). RESULTS: Marked improvement occurred in six patients who had ocular manifestations of Behçet's disease for the first time or with minor damage during their course of chronic relapsing panuveitis. In one patient with advanced ocular Behçet's disease, new relapses were prevented. Retinal infiltrates resolved within 2 weeks; vasculitis, macular oedema, infiltration of the anterior chamber and vitreous resolved within 4 weeks. Mean posterior uveitis score before treatment (nine affected eyes) was 6.6, 4 weeks after IFN it was reduced to 0.4. The mean observation period is 27.6 months, ranging from 14 to 42 months. CONCLUSION: Treatment of ocular symptoms of Behçet's disease with IFN alpha 2a alone or in combination with low dose steroids led to complete remission of ocular vasculitis in all patients treated in this open, uncontrolled trial. Treatment with IFN alpha 2a may prevent permanent retinal or optic nerve damage due to vascular occlusion. No severe side effects occurred. Controlled randomised studies are warranted in order to prove the efficacy of IFN alpha 2a in ocular Behçet's disease and to compare it with other, established treatments such as azathioprine or cyclosporin A.

Adult↗

Acute posterior multifocal placoid pigment epitheliopathy associated with cerebral vasculitis.

Acute multifocal posterior placoid pigment epitheliopathy (APMPPE) is an unusual self-limited retinal disorder that has been associated with various systemic complications. To our knowledge, three prior cases associated with cerebral vasculitis have been described. This article describes a patient with APMPPE and angiographically documented cerebral vasculitis who was notable because of (a) the presence of two different cerebral ischemic events, occurring 1 month apart, and (b) the long latency (3 months) between the onset of ocular symptoms and the second cerebral ischemic event. Recognition of the association between APMPPE and cerebral vasculitis may permit early treatment of CNS involvement and prevention of morbidity.

Adult↗

Ocular features of west nile virus infection in North America: a study of 14 eyes.

PURPOSE: To present a case series of ocular findings of West Nile virus infection (WNVI) in North America. DESIGN: Retrospective, noncomparative, observational case series. PARTICIPANTS: All patients were referred to the authors for WNVI with ocular involvement between the years 2002 and 2005. METHODS: Chart review was performed on all participants. All participants underwent complete ophthalmic evaluation during each examination, including best-corrected Snellen visual acuity measurement, tonometry, slit-lamp biomicroscopy of the anterior and posterior segments, and dilated fundus examination with indirect ophthalmoscopy. Fundus photography and fluorescein angiography were also performed on all eyes. Relevant ocular findings associated with WNVI were recorded and tabulated. MAIN OUTCOME MEASURES: The authors studied the characteristics, frequency, and locations of ocular lesions found in participants' eyes. RESULTS: There were 14 eyes (7 patients) with ocular West Nile virus lesions from 2002 to 2005. Average patient age was 58.4 years (range, 32-85 years). Ocular findings in descending order of frequency included multifocal chorioretinal target lesions in 12 eyes (85.7%), retinal hemorrhages in 7 eyes (50.0%), vitritis in 6 eyes (42.9%), chorioretinal linear streaks in 4 eyes (28.6%), perivascular sheathing and vasculitis in 4 eyes (28.6%), narrowed retinal vessels in 4 eyes (28.6%), disc edema in 4 eyes (28.6%), optic atrophy in 2 eyes (14.3%), vascular occlusion in 2 eyes (14.3%), and VIth nerve palsy in 1 eye (7.1%). Peripheral fundus lesions were found in all 14 eyes (100%), whereas posterior fundus lesions were found in 8 eyes (57.1%). Five patients (71.4%) were diabetic. Diabetic retinopathy was present in 7 eyes (70%). CONCLUSIONS: Multifocal choroiditis is the most common ocular manifestation associated with WNVI, with a typically benign clinical course. Less frequent ocular lesions, including optic neuritis and occlusive vasculitis, frequently induce persistent and likely permanent visual deficit. Diabetic patients and those older than 50 years of age are more vulnerable to the more severe features of WNVI, including more serious ocular lesions.

Adult↗

Orbital infarction syndrome.

BACKGROUND: The authors define orbital infarction as ischemia of all intraorbital and intraocular structures. It is a rare disorder due to the rich anastomotic vascularization of the orbit. PATIENTS: The authors report three patients with orbital infarction with similar clinical presentations: acute blindness, orbital pain, total ophthalmoplegia, and anterior and posterior segment ischemia. RESULTS: Etiologies of orbital infraction syndrome include occlusion of the common carotid artery (case 1), giant-cell arteritis (case 2), and mucormycosis (case 3). CONCLUSION: Three cases discussed in this article illustrate that orbital infarction is a disorder that can occur secondary to different mechanisms such as (1) acute perfusion failure (e.g., common carotid artery occlusion [possibly combined with anomalous orbital anastomotic channels]), 2) systemic vasculitis (e.g., giant-cell arteritis), and (3) orbital cellulitis with vasculitis (mucormycosis). The blindness and retinal and optic nerve damage were permanent, whereas the other signs of orbital ischemia (ophthalmoplegia and anterior segment ischemia) resolved. Therefore, diagnosis of orbital infarction can best be made during the acute phase.

Aged↗

[Ophthalmological aspects of systemic vasculitis].

Ocular findings of systemic vasculitis, such as polyarteritis nodosa, giant cell angitis, and aortitis syndrome were reviewed. Systemic lupus erythematosus is not categorized as systemic vasculitis, however its ocular findings are microangiopathic. Therefore review of its ocular findings was included in this paper. The most common fundus finding in these diseases is ischemic optic neuropathy or retinal vascular occlusions. Therefore several points in diagnosis or pathogenesis of optic neuropathy and retinal and choroidal vaso occlusion were discussed. Choroidal ischemia has come to be able to diagnosed clinically, since fluorescein angiography was applied in these lesions. When choroidal arteries are occluded, overlying retinal pigment epithelium is damaged. This causes disruption of barrier function of the epithelium and allows fluid from choroidal vasculatures to pass into subsensory retinal spaces. This is a pathogenesis of serous detachment of the retina. The retinal arterial occlusion formed non-perfused retina. Such hypoxic retina released angiogenetic factors which stimulate retinal and iris neovascularizations and iris neovascularization may cause neovascular glaucoma.

Blood-Retinal Barrier↗

[Case of the month. Wegener's disease].

A 49 year old woman was admitted because of general poor health condition having lasted for 15 days and been associated for 6 months with sinusitis. During hospitalisation, she developed thrombosis of the retina central artery and signs of cerebral vasculitis. A diagnosis of Wegener disease was suggested based on one side on the presence of multiple excavated pulmonary macronodules and, on the other side, on the signs of cerebral vasculitis. No renal dysfunction was documented in our patient. The detection of serum antineutrophil antibodies (c-ANCA and anti-PR3) confirmed the diagnosis of Wegener disease without the need of surgical pulmonary biopsy. In addition to the typical ENT and pulmonary symptoms, this particular clinical presentation of unusual ocular and cerebral symptoms, reminds us that Wegener disease is a vasculitis that can give multisystemic disorders.

Antibodies, Antineutrophil Cytoplasmic↗

[Vasculitis associated with viral infections].

VIRUSES, THE CAUSE OF VASCULITIS: Although the majority of systemic vasculitis are of unknown causes, the responsibility of a viral infection has been formally demonstrated in some of them and specific treatment can permanently cure them. Each virus incriminated accounts for a particular type of vasculitis. HEPATITIS B VIRAL INFECTION (HBV): Is the cause of polyarteritis nodosa in 36 to 50% of cases. The onset of the symptomatology is acute, usually within a few months following the infection; it is comparable to that observed in the absence of HBV infection. CRYOGLOBULINEMIA RELATED TO THE HEPATITIS C VIRUS (HCV): The clinical manifestations are those of systemic vasculitis with particular tropism for the skin (involvement generally inaugural and almost constant), peripheral nerves and the glomerula. They occur fairly late during the infection. VASCULITIS ASSOCIATED WITH HIV INFECTION: There is strong tropism for the peripheral (multi-neuritis) and central nervous system. During acute parvovirus B19 infection Vasculitis lesions have occasionally been reported following the viremic phase, generally limited to one or several flares of vascular purpura predominating on the lower limbs. FOLLOWING VARICELLA-HERPES ZOSTER INFECTION: Vasculitis occasionally develops in the form of a central neurological deficiency (locomotor deficiency with or without aphasia around one month after an ophthalmologic herpes zoster) or involving the retina or, more rarely, the skin or the kidneys. VASCULITIS ASSOCIATED WITH CYTOMEGALOVIRAL INFECTION: Predominantly observed in immunodepressed patients, vasculitis after CMV infection is diffuse and basically involving the digestive tube, notably the colon, the central nervous system and the skin. A RARE COMPLICATION OF AN HTLV1 INFECTION: Vasculitis of the retina often in the form of necrotic retinitis is often associated with spasmodic paraparessia. THERAPEUTIC STRATEGY: For many vasculitis of viral origin, corticosteroid and immunosuppressive treatments are only indicated in second intention following failure with antiviral agents and the combination of antivirals and plasma exchanges.

Adrenal Cortex Hormones↗

Residues 1-20 of IRBP and whole IRBP elicit different uveitogenic and immunological responses in interferon gamma deficient mice.

Experimental autoimmune uveoretinitis (EAU) is a T-cell-mediated autoimmune disease induced by immunization with uveitogenic retinal antigens, or by the adoptive transfer of uveitogenic T-cells of the Th-1-like phenotype. We have previously shown that IFN-gamma-deficient mice (GKO) on the C57BL/6 background are equally susceptible to interphotoreceptor retinoid binding protein (IRBP)-induced EAU as the wild type (WT). In the present study, we evaluated EAU induction in GKO mice by the newly described H-2(b)epitope contained in residues 1-20 of human IRBP, and compared it to the response to the whole IRBP molecule. Similarly to previous observations with IRBP-induced EAU, delayed type hypersensitivity (DTH) and lymphocyte proliferation responses were elevated in GKO mice, as was production of IL-5 and TNF-alpha. However, unlike the responses induced by whole IRBP, there was no detectable IL-10 production to the peptide. Histopathology on day 21 after immunization, revealed that both GKO and WT mice developed retinal lesions, including damage to the photoreceptor cell layer, vasculitis and inflammatory cellular infiltration, but disease scores were significantly higher in GKO, and retinal detachment was observed only in GKO mice. In contrast to the wild type, the cellular infiltrate in eyes of GKO mice contained a prominent component of eosinophils, although of lower proportion in peptide-induced than in IRBP-induced EAU. We conclude that the cytokine and inflammatory responses to human peptide 1-20 differ perceptibly from the responses to whole bovine IRBP, and may explain the elevated EAU scores of GKO mice compared to wild type.

Animals↗

[Measles retinitis in an immunocompetent child].

BACKGROUND: Measles retinitis is rare in immunocompetent patients and has recently been only sporadically described. This case report reveals the clinical picture, the course of the disease and the prognosis of this entity. HISTORY AND FINDINGS: One month after a diagnosed measles infection, a 12-year-old girl presented with a macular star and peripheral retinal oedema due to a diffuse vasculitis associated with visual impairment in both eyes. Perimetry, electroretinography, and fluorescein angiography revealed a necrotising process along the inflamed vessels. THERAPY AND COURSE: Without therapy vision improved and the macular changes decreased. Some of the paracentral scotomata and a discrete visual acuity defect could still be detected during the follow-up examinations after 2 1/2 and 7 months. CONCLUSIONS: The early diagnosis of measles retinitis by an ophthalmologist can be of great importance because an associated encephalitis could be treated medically if recognized early enough.

Child↗

[Clinical forms and differential diagnosis of angioid streaks of the retina].

The authors describe the clinical forms of complicated angioid streaks (AS) of the retina and the diagnostic symptoms of this condition that help differentiate it from other fundus oculi abnormalities. 52 patients (104 eyes) with retinal AS have been examined. Ophthalmoscopic and angiographic picture permits distinguish 4 clinical forms of complicated retinal AS: exudative hemorrhagic macular changes, non-exudative macular dystrophy, optic nerve atrophy, hemophthalmos. The underlying condition has been differentiated from central involutional retinal dystrophy, complicated myopia, central chorioretinitis, vasculitis. Noteworthy, that complete or partial atrophy of the optic nerve in the presenCe of profuse gastrointestinal hemorrhages is rarely responsible for vision deterioration in the patients with AS.

Adult↗