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Intermediate uveitis.

Intermediate uveitis is an intraocular inflammation involving the anterior vitreous, peripheral retina and pars plana. It usually affects patients from 5 to 30 years old, without gender or racial preferences. The etiology is unknown but there are several associated diseases: multiple sclerosis, idiopathic optic neuritis, autoimmune corneal endotheliopathy, sarcoidosis, thyroid diseases and inflammatory bowel diseases. Symptoms are blurry vision, floaters and distortion of central vision. The syndrome is bilateral in 80% of the patients and chronic with periods of exacerbation and remission. Clinical presentation includes: mild to moderate anterior chamber inflammation, thin keratic precipitates in the inferior portion of the cornea, autoimmune endotheliopathy, vitreitis, vasculitis in the peripheral retina, intravitreal "snowballs," retinal "snowbanking," optic neuritis and cystoid macular edema. Cataract and glaucoma are frequent complications. Treatment of intermediate uveitis is based on periocular and oral corticosteroids. Cryotherapy or laser photocoagulation of the peripheral retina are options in patients with snowbanking when there is an insufficient response to periocular or systemic corticosteroids. Imunosuppression may also be used when other therapies fail, and Cyclosporin A is the first drug of choice. Pars plana vitrectomy is indicated in patients with chronic significant inflammation, non-responsive cystoid macular edema, non-clearing vitreous hemorrhage, tractional retinal detachment and epiretinal membranes. The long-term prognosis of intermediate uveitis is usually good, particularly with strict control of inflammation and with proper management of complications. Patients can often maintain a vision of 20/50 or better.

Humans↗

[Intermediate uveitis].

Intermediate uveitis is a chronic and insidious disease. Children and young adults are preferentially affected. Vision loss and floaters are commonly reported in a quiet eye. The inflammation predominates in the vitreous and some characteristic peripheral exsudates may be observed. Intermediate uveitis is generally idiopathic but may sometimes be associated with a systemic disease that must be researched and treated. The treatment of the idiopathic form is only necessary in case of complications and will consist in corticosteroids eventually combined with immunosuppressive drugs. Surgery (cryotherapy, vitrectomy) will be reserved for complications non responding to medical therapy.

Adolescent↗

[Intermediate uveitis].

Intermediate uveitis defines the group of diseases formerly called pars planitis or chronic cyclitis. Its frequency amounts to 10-20% of all uveitis cases. The anamnesis is vague. The clinical symptoms mainly involve the vitreous. The visual acuity is variable, depending of the involvement of the retina. A slight inflammation of the anterior chamber is possible, but no synechiae are present. The vitreous shows a positive Tyndall and cells, snow balls, eventual plaques on the pars plana. Serious cases are complicated by a retinal vasculitis, which often leads to cystic macular edema and papillary edema. Schisis and retinal holes are more seldom. A complicated cataract can develop. The most valuable complementary tests are fluorescein angiography and electroretinography, useful to detect retinal damage. Aetiological investigations give mostly poor results and are not very useful. Differential diagnosis has to be made, among others, from Fuchs' heterochromic cyclitis and from acute cellular infiltration of the vitreous in case of retinochoroiditis or candida endophthalmitis. Treatment of intermediate uveitis can be only local and discrete when only the vitreous is involved. If the retina is damaged, systemic steroids or immunodepressive cytostatic drugs might be indicated. Despite the duration of the disease, its prognosis remains rather good. Only about 15% of the cases end up with visual impairment to 1/10 or less, while more than half of the cases maintain a visual acuity higher than 0.6, even after three years of disease progress.

Diagnosis, Differential↗

Course and complications of intermediate uveitis.

Intermediate uveitis is associated with a wide variety of complications, particularly in patients attending uveitis referral centres, but little information exists on the frequency of these complications in a general pool of patients. We reviewed all the cases known to have originated in one English county. 86 eyes of 48 patients with a mean follow-up of 48 months were affected. The principal threat to sight was cystoid macular oedema (31%). Other complications included vitreous haemorrhage (8%), disc swelling (5%), periphlebitis (21%) and cataract (5%). Most patients required no treatment other than observation and overall the visual outcome was good with 85% of patients and 63% of eyes having vision of 6/9 or better. Cystoid macular oedema and snowbanking were significantly associated with a worse visual outcome, whilst the other complications were not.

Adolescent↗

[Intermediate uveitis].

Intermediate uveitis is diagnosed in about 10% of the patients seen at the "Uveitis Clinic" of the Second Department of Ophthalmology of Vienna University. Forty-nine patients (with 83 eyes showing signs of the disease) were followed up for between six months and 12 years. Bilateral involvement was seen in 34 patients. The average age of the 27 female patients at the time of diagnosis was 26.2 years, that of the 22 male patients 24.6 years. All the eyes affected showed distinct vitreous opacities; 80% developed exudates overlying the pars plana and 70% showed a mild anterior chamber reaction. Posterior synechiae were found in 10% of the eyes, swelling of the optic disk in 15%, retinal vaso-proliferation in 10%, and dense intravitreal membranes in 6%. Posterior subcapsular cataract, vitreous hemorrhage, retinal detachment, band keratopathy, and atrophy of the eye were rarely seen. The complication most frequently and severely reducing central visual acuity was cystoid macular edema (30%). Whereas 31 patients did not require any treatment over the course of the observation period, it was found that oral corticosteroid therapy led to a significant improvement in vision in 14 patients (20 eyes) with CME. Only few patients required a combination of corticosteroids and Ciclosporin-A as long-term therapy. Vitrectomy and scleral buckling operations were rarely performed.

Adolescent↗

[Fluorophotometry of changes of hemato-ocular barriers in intermediate uveitis].

PURPOSE: Intermediate uveitis presents a characteristic clinical picture with inflammation in the vitreous and peripheral retina. From a physiological point of view, the disease is characterised by a rupture of blood-ocular barriers. To determine the sites involved, a vitreous fluorophotometry was performed in patients with intermediate uveitis and in a group of healthy patients. METHODS: Seventy-one patients with an intermediate uveitis were studied by vitreous fluorophotometry. Anterior chamber and posterior segment fluorophotometric findings were measured 5 and 60 minutes after injection of fluorescein. Results were compared to a group of healthy patients. RESULTS: Vitreous fluorophotometry demonstrated abnormal leakage of fluorescein in all vitreous sectors of the patients suffering from uveitis compared with the control group (p < 0.001). In the anterior chamber, leakage of fluorescein was not significantly different between the two groups (p > 0.05). CONCLUSION: Results obtained suggest that intermediate uveitis involves a generalized permeability disturbance of the posterior segment secondary to a diffuse vasculitis. However, in the anterior segment this disturbance is not found.

Adolescent↗

Systemic diseases associated with intermediate uveitis.

BACKGROUND: Intermediate uveitis is characterized by vitreal inflammation with associated inflammation of the vitreous base and peripheral anterior retina and choroid. It may be found as an isolated and idiopathic condition or in association with systemic disorders such as multiple sclerosis and sarcoidosis. OBJECTIVE: To identify the clinical features of intermediate uveitis and assess its association with systemic diseases. METHODS: Retrospective study of 83 patients presenting with intermediate uveitis between 1970 and 1991. RESULTS: Evidence of systemic disorders was found in 26 of 83 patients (31.3%). Of these 26 patients, 10 had presumed sarcoidosis, 6 had multiple sclerosis, 2 had isolated optic neuritis, 2 had inflammatory bowel disease, 4 had isolated thyroid abnormalities, and 2 had histories suggestive of Epstein-Barr virus infection. Associated ocular findings included cystoid macular edema, peripheral retinal perivascular sheathing, cataracts, posterior vitreous detachment, fine keratic precipitates, preretinal macular fibrosis, retinal tears, retinal detachment, and optic disc edema. CONCLUSIONS: Patients with intermediate uveitis may have associated systemic diseases and should have careful follow-up with regular systemic evaluation.

Adolescent↗

Intermediate uveitis: a hospital based study.

UNLABELLED: Intermediate uveitis affecting pars plana, peripheral retina and underlying choroid affects primarily healthy children and young adults. OBJECTIVE: To study the components of intermediate uveitis at Nepal Eye Hospital in the year 2003 August to 2004 March. MATERIALS AND METHODS: A prospective study was done in patients with intermediate uveitis visiting Nepal Eye Hospital. Sample size was 50. Specially designed proforma of intermediate uveitis was used to collect particulars of the patients, history, clinical examination findings, investigation reports, management protocols used and the final visual outcome. RESULTS: Intermediate uveitis was seen more in 20 to 29 years age group (38%) and more prevalent in males (52%). Among the study population farmers constituted maximum proportion (36%) and Mongoloid origin was mostly affected (30%). High number of patients were from the central development region with highest proportion from Dhading district (34%). Anterior segment affection with keratic precipitates and anterior chamber cells were noted in 72% of involved eyes. Vitreous cells were noted in all affected eyes while 4% had vitreous snow banking and 3% had vitreous snow balls. Most of the patients (52%) received between topical steroid and posterior subtenon injection of Triamcinolone. Visual Acuity was improved in 52% of cases and remained static in 46%.while deteriorated in 2%. CONCLUSION: Significantly high number of patients with intermediate uveitis has been reported from the central development region mainly from Dhading Kathmandu and Nuwakot districts People in their third decade were found to be commonly involved and Mongloid origin were more commonly affected Anterior chamber spill over was seen in higher percentage of patients than described in literature As the patients presented in milder forms visual acuity improved is of cases with the treatment protocol followed.

Adolescent↗

[Ultrasound biomicroscopy in intermediate uveitis].

OBJECTIVE: To investigate the character of intermediate uveitis by ultrasound biomicroscopy (UBM) and determine the potential and usefulness of UBM as a diagnostic procedure in intermediate uveitis. METHODS: Twenty-one cases (35 eyes) of intermediate uveitis were diagnosed by slit lamp biomicroscopy and indirect binocular ophthalmoscopy with scleral indentation. UBM was performed on 35 eyes of 21 patients with intermediate uveitis in order to determine the configuration of pars, plana peripheral retina and vitreous. RESULTS: In 31 of 35 eyes, pathological structures such as spotted or membraneous vitreous condensations, vitreoretinal adhesions with traction on the peripheral retina and ciliary body detachment were identified by UBM. In four eyes, no abnormalities were identified by UBM. CONCLUSION: UBM seems to be a valuable diagnostic technique for the evaluation of patients with intermediate uveitis.

Adolescent↗

Intermediate uveitis.

The cause of intermediate uveitis remains unknown. It is important to rule out other causes of the condition before initiating therapy. Many patients presenting with a mild form of the disease and who have good visual acuity may not require treatment. Those with decreased vision because of inflammation or cystoid macular edema may require periocular injections or systemic administration of corticosteroids (Fig. 2). Those who develop recalcitrant disease and those who experience severe side effects from the steroid therapy may require other immunosuppressive agents. Close monitoring of systemic side effects is required. Laser photocoagulation or cryotherapy of the peripheral retina is useful in patients who develop neovascularization of the vitreous base, in those who are not responsive to periocular injections, and in those who develop severe side effects from corticosteroids. This should be considered before starting systemic immunosuppressive agents. Pars plana vitrectomy with or without cryotherapy or laser photocoagulation is indicated in patients with marked vitreous debris, cystoid macular edema, and in those who develop a vitreous hemorrhage.

Humans↗

Systemic CD4(+) T cell phenotype and activation status in intermediate uveitis.

AIM: To investigate peripheral blood lymphocyte phenotype in patients with intermediate uveitis using CD69, chemokine receptor, and cytokine expression. METHODS: Peripheral blood lymphocytes of 18 patients with idiopathic intermediate uveitis and 6 patients with presumed sarcoid intermediate uveitis were evaluated for CD4(+) T cell expression of CD69, CCR4, CCR5, CXCR3 and the intracellular cytokines IFNgamma, TNFalpha, and interleukin (IL)-10 by flow cytometry, and for IL-2, IL-4, IL-5, IL-10, IFNgamma, and TNFalpha production following unstimulated and activated culture using cytokine bead array and compared with healthy control subjects. RESULTS: The expression of CD69 and TNFalpha by peripheral blood CD4(+) lymphocytes of patients with idiopathic intermediate uveitis and presumed sarcoid intermediate uveitis was significantly higher than control subjects (p = 0.002 and p<0.05, respectively). The ratios of the concentrations of IL-2:IL-5 and IFNgamma:IL-5 in supernatants of activated peripheral blood lymphocyte cultures were significantly higher in patients with presumed sarcoid intermediate uveitis than control subjects. CONCLUSIONS: This study implicates TNFalpha in the pathogenesis of intermediate uveitis, highlighting the potential role of anti-TNF treatments for this disease. Studies of Th1:Th2 cytokine ratios suggested polarisation of the immune response towards Th1 in presumed sarcoid intermediate uveitis despite clinically quiescent systemic disease.

Adolescent↗

The association of HLA-DR15 and intermediate uveitis.

PURPOSE: To evaluate the association between human leukocyte antigen (HLA-DR15) specificity and intermediate uveitis. METHODS: Eighteen patients diagnosed with intermediate uveitis underwent HLA-DR15 serotyping. Additionally, DNA-based phenotyping for a specific HLA-DR15 allele was performed in four patients. The clinical features of HLA-DR15-positive intermediate uveitis were compared with those of HLA-DR15-negative intermediate uveitis. RESULTS: Thirteen of 18 patients (72%) were positive for HLA-DR15. The frequency of the HLA-DR15 specificity in intermediate uveitis patients was significantly higher than in the control subjects (relative risk, 6.36; P < .001). Each of four patients tested carried the specific allele, DR beta 1*1501, which has been associated with multiple sclerosis. In the HLA-DR15-positive group were four patients (31%) with coexisting multiple sclerosis or optic neuritis, one patient with coexisting narcolepsy, and three patients (23%) with a family history of multiple sclerosis. Retinal periphlebitis, especially if bilateral, was a frequent ophthalmoscopic finding in HLA-DR15-positive intermediate uveitis. CONCLUSIONS: This study identifies a significant association between intermediate uveitis and the HLA-DR15 specificity. Patients who are HLA-DR15-positive and have intermediate uveitis may have systemic findings of another HLA-DR15-related disorder. Intermediate uveitis may belong to a constellation of HLA-DR15-related disorders, which includes multiple sclerosis, optic neuritis, and narcolepsy.

Adolescent↗

Elevated serum IL-8 levels are associated with disease activity in idiopathic intermediate uveitis.

AIM: To find a laboratory indicator for systemic involvement in intermediate uveitis. METHODS: Interleukin 8 (IL-8) and C reactive protein (CRP) serum levels were measured in patients with idiopathic intermediate uveitis (n = 61), uveitis controls (n = 143), and normal controls (n = 29). The records of those with intermediate uveitis were reviewed with the emphasis on disease activity and severity as characterised by the presence of cystoid macular oedema, vitreous exudates or snowbank formation, papillitis, and periphlebitis. RESULTS: Increased serum IL-8 (> or = 20 pg/ml) was found in 27 out of 61 patients with intermediate uveitis (p < 0.01), 12 of 27 patients with sarcoid uveitis (p < 0.05), in 19 of 30 patients with HLA-B27 associated acute anterior uveitis (p < 0.05), and in five of 29 healthy controls. Raised IL-8 levels in intermediate uveitis were significantly associated with active disease (p < 0.001) and the presence of vitreous exudates (p < 0.001), papillitis, and periphlebitis (p < 0.01). Elevated CRP levels were found in 12 of the 143 uveitis controls but in none of the intermediate uveitis patients or normal controls. During follow up an associated systemic disease was more frequently noticed in patients with an elevated serum IL-8 at entry into the study. CONCLUSIONS: Elevated IL-8 serum levels were found in patients with active intermediate uveitis of unknown origin. An elevated IL-8 level seems to predispose the patient to a later development of associated systemic disease.

Adult↗

Soluble ICAM-1 serum levels in patients with intermediate uveitis.

AIM: To investigate whether serum levels of soluble intercellular adhesion molecule 1 (sICAM-1) can serve as a marker of the presence of systemic disease in intermediate uveitis. METHODS: In a multicentre study sICAM-1 serum levels were measured in 61 patients with idiopathic intermediate uveitis, controls included 56 uveitis patients with a systemic disease (26 sarcoid associated uveitis and 30 HLA-B27 positive acute anterior uveitis), 58 uveitis patients without systemic disease (30 toxoplasma chorioretinitis and 28 Fuchs' hetrochromic cyclitis), and 21 normal controls. The clinical records of the patients with intermediate uveitis were analysed for disease characteristics at the time of blood sampling and for a relation with the development of a systemic disease after a mean follow up of 4.5 years. RESULTS: Increased serum levels of sICAM-1 were found in 34 out of 61 patients with intermediate uveitis and were significantly different when compared with toxoplasmosis, Fuchs' cyclitis, and healthy controls (p<0.001). Elevated sICAM-1 levels were also found in 18 out of 26 patients with sarcoid uveitis and in 11 out of 30 patients with HLA-B27 associated anterior uveitis. Raised sICAM-1 levels in the intermediate uveitis group were significantly associated with active ocular disease (p<0.01) and the presence of vitreous exudates (p<0.05). Increased levels of sICAM-1 correlated with interleukin 8 levels (IL-8) (tested in a previous study in the same group of intermediate uveitis patients) in patients with active systemic involvement. Follow up of the patients showed that an established or suspected systemic disease was found more often in the 21 intermediate uveitis patients with increased sICAM-1 and IL-8 levels compared with the other 40 patients with intermediate uveitis (p<0.01). CONCLUSIONS: The measurement of both sICAM-1 and IL-8 can be used as a marker for ocular disease activity and for a predisposition of developing an associated systemic disease in intermediate uveitis patients.

Adult↗

Cyclosporine vs tacrolimus therapy for posterior and intermediate uveitis.

OBJECTIVES: To compare the efficacy and tolerability of tacrolimus and cyclosporine therapy for noninfectious posterior segment intraocular inflammation and to evaluate their effect on peripheral blood CD4(+) T-cell phenotype and activation status. METHODS: Thirty-seven patients who required second-line immunosuppression for posterior segment intraocular inflammation were enrolled in this prospective randomized trial of tacrolimus vs cyclosporine therapy. The main outcome measures were visual acuity, binocular indirect ophthalmoscopy score, adverse effects, and quality of life. In addition, peripheral blood CD4(+) T-cell phenotype and activation status were evaluated by flow cytometry before treatment and at 2, 4, and 12 weeks using CD69, chemokine receptor (CCR4, CCR5, and CXCR3), and intracellular cytokine (tumor necrosis factor alpha, interferon-gamma, and interleukin 10) expression. RESULTS: Thirteen patients (68%) taking tacrolimus and 12 patients (67%) taking cyclosporine responded to treatment. Cyclosporine therapy was associated with a higher incidence of reported adverse effects. Mean arterial pressure and serum cholesterol level were significantly higher at 3 months in the cyclosporine group than the tacrolimus group. No significant difference was detected with regard to effect on quality of life or CD4(+) T-cell phenotype. CONCLUSIONS: Tacrolimus and cyclosporine were similar with regard to efficacy for posterior segment intraocular inflammation, but the results suggested a more favorable safety profile for tacrolimus therapy.

Adult↗

Vitreoretinal surgery in intermediate uveitis.

Between 1987 and 1992, vitrectomies were performed in 42 eyes with intermediate uveitis. Pre- and postoperative clinical and ophthalmological parameters were reviewed. Especially the pre- and postoperative time courses of visual acuity were analyzed. We looked for parameters influencing the final visual results. Furthermore, we revised pre- and postoperative recurrent exacerbations of the disease and the duration and dosage of postoperative corticosteroid therapy. The best final visual results were reached in eyes with the best preoperative visual acuities. Anatomic retinal findings and the preoperative duration of intermediate uveitis predominantly influenced the final visual results. Overall, 75% of our patients reached visual acuities of above 20/200. When asked to state their opinion about the final functional result, 80% of the patients were contented. We advocate vitrectomy in patients with intermediate uveitis after intensive follow-up and careful consideration. The anatomic integrity of the retina, a good preoperative visual acuity, and a short preoperative duration of intermediate uveitis are the most important factors influencing the final visual results.

Adolescent↗

Chronic Propionibacterium acnes endophthalmitis as a cause of intermediate uveitis.

Although the pathogenesis in most cases of intermediate uveitis is unknown, a small minority of cases is associated with a variety of specific inflammatory etiologies: sarcoidosis; multiple sclerosis; Lyme disease; syphilis; ocular lymphoma; and as a rare manifestation of Behçet's disease and AIDS. A 61-year-old woman developed pars planitis after cataract surgery. A vitrectomy was performed after ten months when a white capsular plaque and an hypopyon developed. Propionibacterium acnes was isolated. The intermediate uveitis was not controlled until later removal of the intraocular lens and capsular remnants. Chronic propionibacterial endophthalmitis may be a cause of intermediate uveitis.

Cataract Extraction↗

Clinical features and visual outcome of intermediate uveitis in children.

PURPOSE: To determine the presentation, course, treatment and outcome of idiopathic intermediate uveitis in children. METHODS: The records of all patients under the age of 16 presenting with idiopathic intermediate uveitis under the care of one consultant (SL) between 1990 and 2001 were reviewed. The case notes were analysed with respect to presenting symptoms, associated conditions, ocular signs, change in visual acuity, treatment and complications. All patients with systemic disease were specifically excluded from the study. RESULTS: Twenty-six patients (45 eyes) were identified with intermediate uveitis from 114 cases of childhood uveitis seen. The mean age at presentation was 10 years (range 7-13 years) and the average follow-up time was 3 years (range 0-7 years). All patients had varying degrees of vitritis, 28 eyes had snowbanks and 5 had cystoid macular oedema at presentation. Six patients required no treatment, five received topical treatment only, five were managed with orbital floor steroids alone and a further two were managed with orbital floor steroids and oral prednisolone. Systemic steroids were required in eight patients, four of whom also required cyclosporin. Six of these children had successful disease control and maintained a visual acuity of 6/9 in at least one affected eye. CONCLUSION: Idiopathic intermediate uveitis is a relatively uncommon condition in children but can have severe effects on vision. With careful management, vision can be preserved in at least one eye. This study describes the course of the disease in a relatively large series. The variation in disease severity prompts the use of individually tailored immunosuppressive regimens.

Adolescent↗