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[The frosted retinal vascular branch syndrome].

In January 2002, thirty-eight years old healthy man with unusual clinical signs of vasculitis was examined in the Center for Diagnosis and Treatment of Uveitis in our Department of Ophthalmology. The patient had a flu-like disease one month before the signs of the eye disease started. In the beginning of the disease, the visual acuity (VA) was 6/12 (20/40 or 0.5) for distance and Jaeger 8 for near and 6/6 (20/20 or 1.0) and Jaeger 1 respectively. The clinical appearance was similar to this of frosted branch angiitis. The thorough medical examination did not discover link between vasculitis and any systemic disease. Serologic examination discovered positivity of IgG immunoglobulin antibody against cytomegalovirus (CMV) only. The intraocular fluid sampling to confirm CMV antibody presence was denied by the patient. After starting the treatment with gancyclovirus and corticosteroids, the considerable improvement of clinical signs of the frosted branch angiitis was noticed. VA improved after five days of treatment. During one-year follow-up, neither recurrence of vasculitis nor the decline of VA of both eyes was marked. In Czech ophthalmologic literature, we did not find any article referring to frosted branch angiitis, so we took the liberty of offering the Czech term "syndrom omrzlých vĕtví retinálních cév".

Adult↗

Pathologic features of Behçet's syndrome: a review of Japanese autopsy registry data.

The data recorded from 170 autopsies of patients with Behçet's syndrome in Japan during the period from 1961 to 1976 were analyzed. The patients had been in the second to the eighth decade of life, and the ratio of males to females was 5 to 2. A wide spectrum of pathologic findings was observed, with involvement of neurologic, ophthalmic, cardiovascular, pulmonary, gastrointestinal, visceral, genitourinary, and mucocutaneous systems. Some of the common acknowledged clinical features of the syndrome, such as oral ulcers, synovitis, retinal and cutaneous vasculitis, and venous occlusions, were underreported. This apparent discrepancy in an autopsy series may be due to the effects of treatment or the healing process, as well as to the possible incompleteness of the postmortem examination. The accessibility of recorded data in a national autopsy registry offers a unique opportunity for review of the pathologic features of Behçet's syndrome.

Adult↗

[Unusual course of Goodpasture syndrome].

Description of a case of Goodpasture's syndrome with vasculitis of the retinal vessels and macular edema. Unusually, the ocular signs became manifest some time before the appearance of hemoptysis and renal insufficiency.

Adult↗

Modulation of lens-induced uveitis by superoxide dismutase.

Recent studies on reactive oxygen metabolites have suggested that these products may be important mediators in the early tissue damage that develops from immunopathologic inflammations. The superoxide anion appears to be the principal product of the respiratory burst. In order to determine the effects of removing superoxide with superoxide dismutase on modulating the inflammation in experimental phacoanaphylactic endophthalmitis, the animals with this experimental disorder were treated with superoxide dismutase. The treatment resulted in significant reduction of choroidal inflammation, retinal edema and vasculitis, suggesting phlogogenic role of superoxide in the ocular immune-complex disease.

Animals↗

[The eye and chronic inflammatory diseases of the intestines. Immunopathological and genetic aspects. Apropos of 3 cases].

This study tried to approximate the mechanisms of ocular changes in Crohn's disease and ulcerative colitis. First, we reported a series of three patients with ocular complications and chronic inflammatory bowel disease. In all cases, we reported the association of ocular disorders with: various infections: intestinal, extra-intestinal complications, immunological troubles, genetic background: HLA B 27. Second, we reviewed the literature concerning ocular changes in ulcerative colitis and Crohn's disease. Recent immunological developments are studied, regarding: genetic aspects, allergic manifestations. immune complexes and role of IgA antibodies. Practically, uveitis, retinal inflammation or vasculitis can be associated with Crohn's disease and ulcerative colitis. Immunological studies of these patients will allow a better understanding of the mechanisms of the ocular lesions, which remain is complex, multiple and non specific.

Adult↗

Treatment of cytomegalovirus retinopathy in patients with acquired immunodeficiency syndrome. Use of the experimental drug 9-[2-hydroxy-1-(hydroxymethyl)ethoxymethyl]guanine.

Cytomegalovirus (CMV) retinopathy, a relentlessly progressive disease that results in permanent blindness, is the most common opportunistic infection of the eye in patients with the acquired immunodeficiency syndrome. Twenty patients with the acquired immunodeficiency syndrome with CMV retinopathy were treated with a new, experimental, antiviral drug, 9-[2-hydroxy-1-(hydroxymethyl)ethoxymethyl]guanine (BW B759U), in dosages ranging from 5.0 to 14.0 mg/kg/d for a ten- to 20-day course. In 19 patients (95%), treatment halted the progression of infection and decreased retinal opacification, hemorrhage, and vasculitis. Vision remained stable in most cases. Six patients received no additional treatment. Fourteen patients received continued treatment with a lower maintenance dosage. Retinal disease reactivated in all patients who did not receive maintenance therapy immediately after initial treatment, indicating persistence of live virus despite drug therapy. Reactivation of disease also developed in four (40%) of ten patients receiving continuous, uninterrupted maintenance therapy for longer than three weeks. Reversible neutropenia, requiring cessation of treatment, developed in five (25%) of 20 patients on initial treatment and five (36%) of 14 patients receiving maintenance therapy. Rhegmatogenous retinal detachment was a late complication in four patients. BW B759U appears to be useful in the management of CMV retinopathy by reducing or delaying visual loss.

Acquired Immunodeficiency Syndrome↗

[Placoid pigment epitheliopathy and cerebral vasculitis: a clinical case].

We report the case of a 21 year old man who has severe headache and blurred vision since 2 weeks. Ophthalmologic examination discloses typical lesions of acute posterior multifocal placoid pigment epitheliopathy and an homonymous right inferior quadrantanopsia. An inflammatory syndrome and a cerebrospinal fluid lymphocytosis are found. Cerebral imagery is normal. Headache improves only with corticotherapy. We conclude that the neurological attack associated with this acute posterior multifocal placoid pigment epitheliopathy is most likely due to a cerebral vasculitis.

Adult↗

Retinal and choroidal arterial occlusion in Wegener's granulomatosis.

PURPOSE: To describe a patient with Wegener's granulomatosis presenting with bilateral retinal and choroidal arterial occlusion. METHODS: A 64-year-old male with Wegener's granulomatosis was examined using ophthalmoscopy and indocyanine green angiography. RESULTS: The patient had central retinal arterial occlusion in the right eye and branch retinal arterial occlusion in the left eye associated with bilateral choroidal infarcts. Early phase wide-angle ICG angiography of left eye revealed widespread choroidal perfusion problems, presumably from the confluence of multiple segmental arterial occlusions. Indocyanine green angiography of the right eye showed similar widespread choroidal arterial occlusions. CONCLUSIONS: This patient developed loss of vision from multiple arterial occlusions of the retina and choroid. Retinal and choroidal ischemia of this patient may have been caused by vasculitis leading to occlusion of retinal arteries and multiple short posterior ciliary arteries.

Choroid↗

[Bilateral acute retinal necrosis due to herpes simplex virus in inmunocompetent people and acyclovir resistance].

CASE REPORT: A twenty-eight year old woman with necrotitizing retinitis and herpes simplex virus type 1 isolated in aqueous humor with polymerase chain reaction (PCR). An Acyclovir and corticosteroid therapy was started with unsuccessful response, Foscarnet was added getting quiescence of lesions. DISCUSSION: Acute Retinal Necrosis Syndrome (ARNS), induced by a virus of the herpes family, could develop in immunocompetent people. A characteristic clinical case with uveitis and vitritis, white retinitis areas and occlusive vasculitis is reported. Antiviral therapy with acyclovir and antiinflammatory treatment must be established quickly. Foscarnet can effectively treat ARNS in inmunocompetent patients. In spite of therapy, this is a potentially blinding retinal disease.

Acute Disease↗

Remission of Behcet's disease with anti-tumor necrosis factor monoclonal antibody therapy: a case report.

BACKGROUND: Behcet's disease (BD) is a chronic relapsing multisystem inflammatory disorder with mucocutaneous, ocular, articular, vascular, gastrointestinal and central nervous system manifestations. Tumor necrosis factor (TNF)-alpha is believed to play a pivotal role in BD. Therapeutic blockade of the activity of TNF has been successfully given in a short course of therapy with favorable effects in patients with BD refractory to conventional immunosuppressive drugs. We aimed to find out whether a 12-month treatment with infliximab, a chimeric monoclonal antibody to TNF-alpha, had any beneficial effect in reducing relapses of a patient with long-standing BD refractory to conventional immunosuppressive drugs. CASE PRESENTATION: A 54 year-old-woman with a 35-year history of BD with orogenital ulcerations, arthritis in the right knee and retinal lesions compatible with vasculitis received infliximab, 5 mg/kg by a two-hour intravenous infusion. Symptoms improved within 24 hours and eight days later the genital and oral ulcers healed as well as the arthritis in the right knee subsided. The retinal infiltrates completely resolved within 10 days. The infusions were repeated at weeks 2, 6, 14, 22 and then every 8 weeks. The patient was able to return to her domestic daily life. No exacerbation of the mucocutaneous ocular or arthritic symptoms occurred during the treatment period. CONCLUSIONS: Previous studies have suggested that infliximab given in a short course of treatment is effective in inducing remission of severe mucocutaneous, gastrointestinal and ocular manifestations of BD. Our patient received a 12-month infliximab treatment showing a favorable effect on remission of BD manifestations. The long-term infliximab treatment appears as a new therapeutic option for patients with active BD who failed to respond to conventional immunosuppressive agents.

Antibodies, Monoclonal↗

Indocyanine green angiography of acute multifocal posterior placoid pigment epitheliopathy.

PURPOSE: To understand the pathophysiology of acute multifocal posterior placoid pigment epitheliopathy (AMPPPE). METHODS: Indocyanine green (ICG) angiography was used for three patients, four eyes with acute AMPPPE and four eyes with healed AMPPPE. RESULTS: The angiographic photographs demonstrated widespread choroidal hypofluorescence of the active and healed lesions. CONCLUSIONS: Although the pathogenesis and histopathology of AMPPPE are unknown, the literature suggests that AMPPPE may represent an inflammatory reaction, possibly a delayed-type hypersensitivity reaction. The ICG choroidal hypofluorescence in AMPPPE is probably due to partial choroidal vascular occlusion secondary to occlusive vasculitis.

Acute Disease↗

Multifocal chorioretinal atrophy associated with herpes zoster ophthalmicus.

A 73-year-old woman developed multiple depigmented lesions in the fundus 4-6 months after an episode of acute Herpes zoster ophthalmicus. Post-mortem examination of the globe 15 years after this acute episode confirmed multiple old chorioretinal scars probably due to vasculitis of the short posterior ciliary arteries and branches. Patchy old infarcts were also noted in the iris.

Acute Disease↗