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[Cystic fibrosis and uveitis in childhood and multiple sclerosis at adulthood].

We report the case of a patient with cystic fibrosis who suffered severe bilateral uveitis, with hypopyon, retinal vasculitis, and polyarthritis when he was 6 years old. No etiology could be found. Multiple sclerosis began when he was 22 years old. This panuveitis was clinically very different from the uveitis usually associated with multiple sclerosis. Polyarthritis and skin vasculitis have been reported in the course of cystic fibrosis, but no uveitis nor retinal vasculitis have been described. This raises the question of the role of multiple sclerosis and cystic fibrosis in the pathogenesis of this case of uveitis.

Adult↗

Late developing lesions in birdshot retinochoroidopathy.

Birdshot retinochoroidopathy is characterized by depigmented spots radiating from the optic disk in association with mild vitritis, retinal vasculitis, and involvement of the optic nerve head. In two patients, we traced the long-term course of uveitis with vitritis, retinal vasculitis, and papillitis that resulted in the typical cream-colored spots of birdshot retinochoroidopathy after seven and eight years, respectively, of follow-up. These observations suggest that in long-standing inflammation of the retinal vasculature and uveal tract, the HLA-A29 antigen should be assessed, because the development of typical lesions of birdshot retinochoroidopathy may be delayed in some patients.

Adult↗

[Cytomegalovirus retinitis associated with Acquired immunodeficiency syndrome].

OBJECTIVE: To investigate the fundus characteristics, systemic features and therapeutic outcomes of cytomegalovirus (CMV) retinitis associated with acquired immunodeficiency syndrome (AIDS). METHODS: Fundus features, visual acuity, fundus fluorescence angiography (FFA) and CD4(+)T-lymphocyte counts of 15 eyes (8 patients) of CMV retinitis associated with AIDS were analyzed. The time of average follow-up was 16 months. Intravitreal injection of ganciclovir (400 microg) was performed in 4 eyes (2 patients). RESULTS: In the initial examination, visual acuity of the patients was as following: < or = 0.2, 10 eyes (66.7%); no light perception (NLP) (2 eyes); light perception (LP) (2 eyes); 0.04-0.2, 6 eyes; > or = 1.0, 3 eyes (20.0%) and 0.8 or 0.9 each for 1 eye (13.3%). The fundus manifestation includes: the retinal vasculitis; dense, full-thickness, yellow-white lesions along vascular distribution, and hemorrhage on the retinal surface; granular with irregular border featured as "cheese and ketchup retinitis" was revealed in 12 eyes; the vitreous was clear or light opaque. Late stage of the retinopathy was demonstrated in 2 eyes characterized as grayish atrophic retina, vessel-sclerotic and attenuated, retinal pigment epithelium (RPE) atrophy, prominent choroid vasculature, and optic nerve atrophy. Retinal detachment was found in 1 eye. CD4(+)T-lymphocytes counts in peripheral blood were between 0 approximately 36/mm(3), average (15.0 +/- 12.9) mm(3). Visual acuity improved, fundus lesions disappeared observably and hemorrhage was absorbed in the 4 eyes after intravitreal injection of ganciclovir. CONCLUSIONS: CMV retinitis is the severest and the most common intraocular complication in patients with AIDS. For the patients with yellow-white retinal lesions, hemorrhage and retinal vasculitis of undefined cause, the antibody of serum anti-human immunodeficiency virus (HIV) should be screened. Routing eye examination should be performed if the serological test of HIV is positive.

Acquired Immunodeficiency Syndrome↗

Severe retinochoroidopathy: variations of humoral and cellular immunity to S-antigen in a longitudinal study.

Ten patients with birdshot retinochoroidopathy, six with isolated retinal vasculitis and eight with Behçet's disease were treated with cyclosporine for one to three years. Autoantibodies to several retinal proteins, circulating lymphocyte subsets and cellular reactivity to S-antigen were evaluated repeatedly during this period. Autoantibody titers were similar in patients and in controls. However the serum content of antibodies to IRBP or S-antigen was lessened during inflammatory periods in some patients. In some sera, antibodies reacted with enzyme digested S-antigen preparations by immunoblot, whereas the same sera were negative for the native protein. A decrease of the CD4+ subpopulation of peripheral blood lymphocytes was associated with relapses of ocular inflammation in birdshot retinochoroidopathy. In this disease and in idiopathic retinal vasculitis, the positive lymphocyte stimulation test and basophil degranulation test with S-antigen were significantly most frequent in the period preceding a relapse of ocular inflammation. These tests could therefore be of predictive value for relapses occurring within the next few months.

Adult↗

Neovascularisation associated with posterior uveitis.

Twenty-six patients (39 eyes) with retinal neovascularisation associated with ocular inflammation were identified from the retinal vasculitis clinic at St Thomas's Hospital. Eight patients had sarcoidosis, seven patients Behçet's disease, and 11 had idiopathic retinal vasculitis. Twenty-three patients had required systemic therapy to control the inflammation and 11 patients received laser photocoagulation. Fluorescein angiography showed significant capillary closure in 15 eyes and diffuse microvascular leakage in the remaining 24 eyes. All patients had posterior vitreous detachment. The visual prognosis was good despite vitreous haemorrhage being the presenting feature in 22 eyes, and the new vessels resolved in 70% of cases. However, laser treatment was followed by a significant increase in cystoid macular oedema (p less than 0.01). This retrospective study suggests that medical therapy is the first line of treatment in this group of patients. Photocoagulation should be performed when the eye is quiet and should be reserved for patients with recurrent vitreous haemorrhages and significant capillary closure.

Adolescent↗

A new case of acute idiopathic frosted branch angiitis in Europe.

CASE REPORT: A 20-year-old male with no history of any systemic or eye disease developed loss of visual acuity in both eyes. White exudates surrounding the retinal veins from the posterior pole to the periphery, retinal edema and hemorrhages in both eyes were evident on ophthalmoscopy. Fluorescein angiography showed leakage of the dye from the veins and extensive staining of the vein walls. A diagnosis of acute frosted branch angiitis was made. Systemic examination revealed axillary, submandibular and inguinal lymphadenopathies. VCA IgM antibody for Epstein-Barr virus was negative and IgG positive. Biopsy was taken of an axillary lymph node; a non-specific inflammatory reaction was found on anatomopathologic study. The patient was started on 90 mg prednisolone daily. After two weeks retinal vasculitis had improved and the lymphadenopathies soon resolved. Small, hard exudates were present in the posterior pole during absorption of the retinal edema and resolution of the vascular inflammation. Systemic prednisolone were reduced progressively and definitively withdrawn two months later. The patient's visual acuity was 20/25 in both eyes. No fibrotic scar tissue or atrophic lesions were noted in either fundus. CONCLUSIONS: We report a new case of acute frosted branch angiitis with an onset and favorable clinical course similar to previous reports. We found the additional presence of lymphadenopathies but have been unable to establish a possible causal agent. To our knowledge, apart from a recent case of frosted branch angiitis-like response in Greece, the present case is the first reported in western Europe.

Acute Disease↗

Experimental posterior uveitis. I: A clinical, angiographic, and pathological study.

The clinical, angiographic, and histopathological features of experimental posterior uveitis in the black hooded Lister rat are described. This mild form of experimental allergic uveoretinitis (EAU) is induced by sensitisation with retinal S antigen in Freund's complete adjuvant, and the inflammation produced is confined to the posterior segment of the eye. This allows for the first time precise photographic and angiographic documentation of the evolution of clinical signs, because there is minimal clouding of the vitreous by inflammatory cells. Clinically the disease is characterised by the appearance of disc oedema and periphlebitis, followed by focal infiltrates in the deep retinal layers, with eventual atrophy of the pigment epithelium. Histologically, retinal vasculitis is associated with focal mononuclear cell infiltration and necrosis of the photoreceptor layers. This model closely resembles the clinical features of idiopathic retinal vasculitis seen in man.

Animals↗

Ocular involvement in patients infected by the West Nile virus.

West Nile virus (WNV), a mosquito-borne RNA virus for which there is no treatment, began emerging as a threat to health in the United States in 1999. Since then, its frequency and apparent clinical severity have increased. Patients with severe disease may experience ocular complications that include pain, vitreous inflammation, nonrelapsing chorioretinitis, retinal vasculitis, chorioretinal scarring, optic neuritis, and retinal hemorrhages. Age appears to be a risk factor for the development of ocular symptoms. Most patients with ocular involvement report floaters or decreased vision. Many ocular inflammatory conditions with an associated underlying systemic cause often present with chorioretinitis. However, the characteristic distribution and linear array or scattered pattern of the chorioretinal lesions seen in WNV is distinctive. The foregoing notwithstanding, other multifocal choroidopathies must be considered including syphilis, ocular histoplasmosis, multifocal choroiditis, tuberculosis, and sarcoid. Although topical steroids were reported to resolve WNV-induced uveitis and its associated keratic precipitates, most cases resolved irrespective of treatment, and relapses were uncommon. It is important for the eye care professional to be alert to the possible presence of WNV, particularly in older patients who present with ocular symptoms during mosquito season. Thus, a thorough ocular evaluation should include a dilated fundus examination and, when indicated, fluorescein angiography should be performed in patients suspected of being infected with WNV.

Age Factors↗

Association of complement allotype C4B2 with anterior uveitis.

We have studied allotypes of the fourth component of complement (C4) in 44 patients with inflammatory eye disease in order to define genetic susceptibility factors further. Twenty-six patients had uveitis (18 had anterior uveitis) and 18 patients had retinal vasculitis. There was an increased incidence of the C4B2 allotype in patients with anterior uveitis (pc less than 0.002), especially in HLA B27 positive males. In contrast, there was no increased incidence of specific allotypes in patients with posterior uveitis or retinal vasculitis. This genetic association may form part of a disease susceptibility supratype in patients with anterior uveitis.

Alleles↗

Macular ischaemia in posterior uveitis.

The commonest cause of visual morbidity in patients with posterior uveitis is cystoid macular oedema, which usually responds to immunosuppressive treatment. However, a small group of patients do not have a satisfactory visual outcome despite apparently adequate therapy. In a retrospective study of 345 angiograms of 135 patients with active non-occlusive retinal vasculitis 12 patients were identified by independent masked review as showing macular ischaemia on their fluorescein angiograms. Four patients had Behçet's disease, 4 sarcoidosis, and 4 idiopathic retinal vasculitis. Follow-up of these patients for an average of 36 months (range 6-120 months) showed that visual acuity failed to improve in 4 patients and dropped by an average of three lines Snellen in the other 8. We suggest that a poor visual outcome in some patients with posterior uveitis may be predicted by the presence of macular ischaemia on fluorescein angiography and that immunosuppressive therapy should be prescribed with caution in these patients.

Adult↗

[Retinal arteritis in a case of sarcoidosis. A case report].

Retinal vasculitis is a common finding and occurs in 10% - 83% of patients with sarcoidosis. In most cases, there are bilateral, peripheral and nonischemic periphlebitis. In contrast, periarterial involvement is rarely observed. We report a case of retinal vasculitis associated with sarcoidosis and its differential diagnosis. Sarcoidosis should not be excluded in case of periarteritis.

Aged↗

[Serum anti-retinal antibodies and intensity of endogenous posterior uveitis ].

PURPOSE: To analyse the correlation between the anti-retinal antibodies (ARA) and intensity of intraocular inflammation in patients with endogenous posterior uveitis. MATERIAL AND METHODS: 50 patients--29 women (58%) and 21 men (42%) in age 15-70 yrs with idiopathic posterior uveitis were examined. Intraocular inflammation intensity was scored on standard uveitis grading system prepared by BenEzra et al. In all cases the ARA were determined by indirect immunofluorescence test on normal monkey retina as a substrate and FITC-labelled goat's anti-human IgA, G, M serum (Euroimmun-Germany). The control serum was obtained from 50 blood donors: 20 women (40%) and 30 men (60%) in age 15-68 yrs. RESULTS: The statistical analysis showed a correlation between the ARA serum levels and intraocular inflammation intensity. High correlation was found in first control between serum ARA level and the degree of vitreous inflammation and the presence of fresh vessels sheathing, characteristic for retinal vasculitis. In next three controls, this correlation was still present, but only in a case of vitreous changes. And at the end of observation no correlation was found, neither between serum ARA levels and the activity of vitreous inflammation, nor between the ARA levels and retinal vasculitis intensity. CONCLUSIONS: The assessment of serum ARA in patients with idiopathic posterior uveitis can act as an indicator for intraocular inflammation intensity and can reflect the retinal autoimmunity.

Adolescent↗

Severe periphlebitis, peripheral retinal ischemia, and preretinal neovascularization in patients with multiple sclerosis.

Two patients with definite multiple sclerosis and marked retinal periphlebitis developed occlusive peripheral retinal vasculitis, which resulted in peripheral retinal ischemia and peripheral retinal neovascularization. Results of investigation for other causes of peripheral proliferative retinopathies were negative in both patients although one patient had a positive anticardiolipin antibody. Both patients have been followed up for over seven years and have maintained good visual acuity with mild regression of the preretinal neovascularization without laser intervention. An analysis of these two cases and six other reported cases indicates that severe periphlebitis can evolve into occlusive peripheral vasculitis, which results in peripheral retinal neovascularization in patients with multiple sclerosis.

Adult↗

Trans-Tenon's retrobulbar triamcinolone infusion for the treatment of uveitis.

AIM: To assess efficacy and complications of trans-Tenon's retrobulbar infusion of triamcinolone acetonide for posterior uveitic inflammation. METHODS: Non-randomised, uncontrolled, retrospective study of 51 eyes of 37 patients who underwent triamcinolone infusion for vitritis, cystoid macular oedema (CMO), or posterior retinal vasculitis using a long blunt cannula via an incision made through conjunctiva and Tenon's capsule. RESULTS: Overall clinical efficacy was 86%; 96% for vitritis, 82% for CMO, and 33% for posterior retinal vasculitis. Mean visual acuity improved within 1 month after triamcinolone infusion (p <0.05). Cataract progression and intraocular pressure elevation were observed in 31% and 27% of eyes, respectively. CONCLUSION: Trans-Tenon's retrobulbar triamcinolone infusion may be a safe and effective treatment for posterior uveitic inflammation.

Adult↗

Ocular syphilis: the new epidemic.

AIM: To present the clinical presentation, diagnosis, and management of syphilitic uveitis in the context of an epidemic of syphilis in the UK. METHOD: Retrospective clinical case series. RESULTS: Six new cases of syphilitic uveitis presented to the Manchester Uveitis Clinic in 2004, after a 15-fold increase in the incidence of syphilis in the UK, including 615 cases in Greater Manchester in the 5 years to 2004. Four cases had secondary syphilis, two had latent disease, two had no rash, and two were HIV positive. Ocular involvement included anterior or panuveitis, retinitis, retinal vasculitis, and papillitis. All resolved on treatment including intramuscular procaine penicillin G with oral probenecid. CONCLUSIONS: Syphilis is much more common recently and syphilitic uveitis should be considered in all patients with rash and/or headache, where there is retinitis and/or retinal vasculitis, or in any uveitis of uncertain origin. Treatment is that of neurosyphilis.

Adult↗

Chronic infantile neurological cutaneous and articular syndrome: two new cases with rare manifestations.

UNLABELLED: CINCA/IOMID is a systemic inflammatory disorder of unknown aetiology that resembles congenital infection and systemic juvenile chronic arthritis (JCA). This disorder is characterized by neonatal onset, persistent rash, ocular inflammatory lesions, and progressive articular and neurological involvement. We report two new patients with this syndrome. Both children presented periodic bouts of cutaneous rash, fever, organomegaly, articular involvement with typical radiological features, and developmental delay. One of the patients presented neonatal jaundice and elevation of liver enzymes; inflammatory infiltrates were observed in the liver biopsy. The other patient showed retinal vasculitis detected at age 18 mo on fundoscopy and fluorescent angiography. Therapy with azathioprine was associated with prolonged remission of this complication. In both cases, the disease was diagnosed after some delay. CONCLUSION: Early hepatitis and retinal vasculitis are rare features of CINCA/IOMID that may help differentiate this syndrome from JRA. Azathioprine may have induced the remission of vasculitis in one case.

Abnormalities, Multiple↗

Visual loss with dancing extremities and mental disturbances.

A case is reported of a teenage girl, who presented with a profound loss of vision in the right eye, secondary to retinal vasculitis. During the preceding year, a gradual change in her personality had been noted, associated with a deterioration in her intellect. She developed an area of pigment epithelial disturbance in the macular region of the right eye, and subsequently, optic atrophy. One year after this, she had a similar, but more localized episode in the left eye. She later developed involuntary movements, and two years after initial presentation, a diagnosis of subacute sclerosing panencephalitis was made. Subsequently her clinical condition deteriorated rapidly and she died. Post-mortem examination confirmed the diagnosis of subacute sclerosing panencephalitis. This case demonstrates the insidious nature of the disease and suggests that the diagnosis of subacute sclerosing panencephalitis should be considered in any child or young adult who presents with unexplained retinal vasculitis, maculopathy or chorioretinitis.

Adolescent↗

The efficacy of cyclosporin-a in the treatment of Behçet's disease.

We used cyclosporin A (CA) to treat 14 patients (25 eyes) with Behçet's disease characterized by severe retinal vasculitis and active intraocular inflammation. All of the patients had been treated previously with corticosteroids, colchicine, and immunosuppressives, without satisfactory results. The patients were given an initial oral dose of 5 mg/kg/day. All medication was tapered and eventually completely stopped after 12 months. The number and severity of ocular attacks (anterior and/or posterior uveitis with vitreous haze) were significantly reduced below pretreatment levels during therapy (Kolmogorov Smirnov two-pair test: P < .05). Visual acuity improved in 32% and remained unchanged in 44%. No ocular attacks recurred in 11 eyes; they recurred 1 time in 8 eyes; 2 times in 3 eyes; and 3 times in 3 eyes during CA therapy. There were no significant changes in the level of retinal vasculitis. In two cases with vaso-obstructive changes, treatment with CA was not effective and laser photocoagulation was performed. Based on our study, we recommend that an initial dose of 5 mg/kg/day CA be used in the systemic medical treatment of ocular Behçet's disease. The CA can be continued at this low dosage for an unspecified time. If intraocular inflammation does not totally resolve at this dosage or the inflammatory process recurs, combining the CA with low doses of a steroid should be considered.

Administration, Oral↗