Intermediate uveitis: comparison between childhood-onset and adult-onset disease.

PURPOSE: To compare demographic and clinical data, systemic disease association, visual prognosis, and complications found in childhood and adult onset of intermediate uveitis (IU). METHODS: Retrospective cohort study of 287 patients with IU: 122 (42.5%) children at onset (<16 years), 165 (57.5%) adults. The data were entered on a computer-based standardized data entry form for statistical analysis. Student t test was used regarding differences in means. To assess significance of differences in proportions, we used the χ2 test, but when the population in the subgroup was ≤ 5 patients, we used the Fisher exact test (p<0.05 were deemed to be statistically significant and as reported were not corrected for multiple testing and so should be viewed as nominal). RESULTS: A total of 61% of childhood-onset cases occurred in boys (44% in the adult group), while adult onset was more common in women (56% vs 39%) (p = 0.004, odds ratio [OR] 2.06). Regardless of the onset age, IU was frequently bilateral and idiopathic. The most frequent complication was cystoid macular edema (27.5%, p = 0.469, OR 0.85), which was also the most frequent cause of visual loss. Optic disc edema was more prevalent in children (15.5% vs 9.2%; p = 0.027, OR 1.81), ocular hypertension in adults (15.6% vs 7.5%, p = 0.007, OR 0.44). A total of 25 patients had non-idiopathic IU. A total of 14 patients had multiple sclerosis. CONCLUSIONS: Intermediate uveitis was mostly idiopathic and bilateral in both groups. Visual prognosis was good and it was not age-related. Childhood onset was more frequent in boys, adult onset in women. Endocrinal factors could be involved.

Intermediate uveitis in a pediatric Italian population.

PURPOSE: To investigate clinical data, outcome, and treatment of intermediate uveitis in children. METHODS: Retrospective cohort study, including 116 children affected by intermediate uveitis. RESULTS: Sixty-two percent of patients were males. Ocular involvement was bilateral in 84%. Mean age at uveitis onset was 10.3 ± 3.6 years and 12.2 ± 7 years at first visit. Ninety-seven percent were classified as having idiopathic uveitis. The incidence of ocular complications during follow-up was 0.131/eye-year. Macular edema or a marked vitreous haze at presentation may be risks factors for incident visual loss. The most frequent causes of visual loss were macular edema (64%) and cataract (21%). CONCLUSIONS: Most cases are idiopathic (pars planitis); only few children presented associated systemic disease. Macular edema and severe vitritis at presentation are associated with increased risk of visual impairment. Cystoid macular edema is the main cause of visual impairment.

Role of the intravitreal growth factors in the pathogenesis of idiopathic epiretinal membrane.

PURPOSE: The aim of the present study is to evaluate the roles of TGFs ß1 and ß2, glial cell line-derived neurotrophic factor (GDNF), and nerve growth factor (NGF) in the pathogenesis of idiopathic epiretinal membrane (ERM). METHODS: Eight patients, six males and two females, with an average age of 60.25 ± 17.16 years (range, 33-75 years) who were affected by idiopathic ERM were enrolled in the study. All patients underwent standard pars plana vitrectomy surgery with membrane removal and specific ELISA was performed to evaluate TGFß1, TGFß2, GDNF, and NGF in the vitreous samples. This was repeated after acidification of the samples with hydrochloric acid. RESULTS: Before acidification, ELISA analysis revealed a significant increase of TGFß2 in the samples with idiopathic ERM (327.98 ± 99.58 pg/mL; range, 206.864-466.235 pg/mL) compared to the control group (187.17 ± 58.20 pg/mL; range, 132.758-271.707 pg/mL; t = 3.4; P < 0.05). A statistically significant difference was also obtained after acidification of the samples (618.15 ± 201.43 pg/mL; range, 409.795-866.215 pg/mL compared to 265.04 ± 98.15 pg/mL; range, 152.478-352.101 pg/mL; t = 4.5; P < 0.05). Notably, before acidification the differences in NGF between the two groups were not statistically significant (t = 0.79; P = 0.46), while after acidification a significant increase of the NGF levels in ERM samples was found in comparison with the control group (723.41 ± 235.4 vs. 242.84 ± 104.61; t = 3; P < 0.05). CONCLUSIONS: The present study reveals that TGFß2 and NGF are associated with idiopathic ERMs, suggesting a novel compensatory mechanism so far never proposed.

Central serous chorioretinopathy as a presenting symptom of endogenous Cushing syndrome: a case report.

PURPOSE: To report a case of Cushing syndrome due to adrenocortical adenoma revealed by central serous chorioretinopathy. MATERIALS AND METHODS: A 45-year-old man presented with blurred vision and metamorphopsia in the left eye. He reported few episodes of high blood pressure in the last 3 months. RESULTS: Visual acuity was 20/40 in the left eye. Fundus oculi examination revealed central serous chorioretinopathy in the left eye. Grade 1 hypertension was found. Increased serum and urinary levels of cortisol and reduced serum levels of ACTH were observed. Diagnosis of Cushing syndrome was made. Computed tomography scan revealed a right adrenal mass that was surgically removed; histologic examination showed an adrenocortical adenoma. Three months after surgical treatment, visual acuity improved to 20/20 and central serous chorioretinopathy completely resolved. CONCLUSIONS: Central serous chorioretinopathy may be the presenting symptom of Cushing syndrome in a patient with adrenocortical adenoma.

Retinal complications of juvenile idiopathic arthritis-related uveitis: a microperimetry and optical coherence tomography study.

PURPOSE: To evaluate ocular complications of juvenile idiopathic arthritis (JIA)-related uveitis; to study macular morphology and sensitivity. METHODS: Retrospective chart review of 67 patients. Fourteen consecutive patients (24 eyes) observed from January to December 2008 were submitted to optical coherence tomography study (OCT) and microperimetry MP-1 examination. RESULTS: Most frequent complications were posterior synechiae (63.0%), band keratopathy (53.9%), and cataract (31.8%). Posterior complications were documented in 37.0% of eyes: 13.8% macular edema, 12.0% papillitis, 6.8% epiretinal membranes, 2.5% retinal vasculitis, 1.7% retinal detachment. At OCT examination 25.0% of eyes presented macular edema; macular sensitivity by MP-1 was < or = 16.5 dB in 25.0% of eyes. CONCLUSIONS: Visual prognosis is good despite ocular complications. Incidence of macular edema seems to be higher when using OCT. Microperimetry may represent a useful tool in detecting alteration in retinal sensitivity that may supplement visual acuity in the follow-up of macular edema.

Optical coherence tomography for classification and clinical evaluation of macular edema in patients with uveitis.

PURPOSE: To study use of optical coherence tomography (OCT) for macular edema in uveitis. METHODS: In total, 44 patients with uveitis and macular edema underwent OCT. Data obtained were correlated with anatomic type, duration of uveitis, and visual acuity (VA). RESULTS: Both cystoid macular edema (CME) and diffuse macular edema (DME) correlate negatively with VA, although statistically CME showed stronger correlation (p = .02). Also CME had higher mean foveal thickness than DME (p < .01). Negative correlation between foveal thickness and VA (p < .05) was observed. CME correlates with uveitis duration (p < .01). CONCLUSIONS: CME and macular thickening correlate with VA reduction and uveitis duration.

Infliximab treatment for ocular and extraocular manifestations of Behçet's disease.

PURPOSE: To assess the efficacy and safety of infliximab in the treatment of sight-threatening uveitis and extraocular manifestations in patients with Behçet's disease. METHODS: Twelve patients with Behçet's disease and uveitis were treated with infliximab after unsuccessful therapy with other immunosuppressive drugs. The main outcome measures were as follows: the number of uveitis relapses, the number of Behçet's disease-related extraocular lesions, and the amount of corticosteroids administered during the treatment as well as during an equal prior period of time while the patients were on other immunosuppressive agents. Visual acuity was recorded at the beginning of infliximab therapy and at the end of follow-up, and was defined as stable if it did not change from baseline, increased if it showed at least one line of improvement from baseline, and decreased if it showed at least a one line decrease from baseline. RESULTS: During an average follow-up of 16.67 +/- 7.63 months (median, 15 months), 11 patients (91.6%) showed a reduction in the number of ocular relapses (relapse/month, from 0.35 +/- 0.17 to 0.12 +/- 0.17, P < 0.001). All of the patients (n = 11) who were taking corticosteroids before infliximab were able to reduce the amount of corticosteroids taken daily during infliximab treatment (from 24.33 +/- 10.84 mg/prednisone per day to 8.97 +/- 6.81 mg/prednisone per day, P < 0.001), and all presented with a reduced onset of extraocular manifestations of Behçet's disease (mean total number, from 2.83 +/- 3.61 to 1.51 +/- 2.35, P = 0.039). One patient, who had to stop treatment 2 months after starting because of the onset of pulmonary tuberculosis, showed the same number of relapses during infliximab treatment but was able to reduce the mean daily corticosteroid dose. Visual acuity increased by one or more lines in three eyes (12.5%) and remained unchanged in 87.5% of the eyes. Infliximab-related side effects appeared in four patients (33.3%). CONCLUSIONS: Infliximab was effective in the treatment of uveitis in these Behçet's disease patients, significantly reducing the number of ocular relapses and making possible a significant reduction in the daily dose of corticosteroids administered. Extraocular manifestations of Behçet's disease were also controlled by infliximab. Nevertheless, side effects were not uncommon, and an extensive study of systemic conditions before infliximab administration had to be carried out to exclude systemic infection, particularly prior tuberculosis.

Paracentral corneal melting in a patient with Vogt-Koyanagi-Harada's syndrome, psoriasis, and Hashimoto's thyroiditis.

The paper describes the occurrence of unilateral paracentral corneal melting in a 65-year-old woman with ocular and systemic signs of Vogt-Koyanagi-Harada's syndrome (VKH), psoriasis, and Hashimoto's thyroiditis. Corneal melting, often occurring in association with systemic vasculitis and connective tissue diseases, has rarely been reported in relation to diffuse psoriasis and never in association with VKH. A possible relationship between corneal melting and VKH is suggested.

Uveitis in Turner's syndrome.

BACKGROUND: Turner's syndrome is a chromosomal abnormality where phenotypic females have either a missing X chromosome or a structural aberration of the X chromosome. Several ocular diseases have been associated with Turner's syndrome in the past, including one case only of proven iridocyclitis. METHODS: In this study we report the clinical findings of three females with Turner's syndrome and uveitis followed up for a mean period of 12.6 months. Two were observed in childhood or adolescence (10 and 16 years old). RESULTS: All the patients showed iridocyclitis, in one case complicated by the onset of papilledema and cystoid macular edema. Associated systemic diseases (psoriasis and juvenile seronegative arthritis) were diagnosed in two cases. The third patient showed positive antinuclear antibody and HLA-DR11, without any clinical or radiologic signs of arthritis. The iridocyclitis tended to become chronic with time, and this may be partially due to the endocrinologic contraindications to the use of systemic steroids. Nevertheless, the final visual acuity was > or =8/10 in all three cases. CONCLUSIONS: Iridocyclitis should be included in the list of ocular manifestations in Turner's syndrome. It may tend to become chronic and may be found especially in those patients presenting other associated autoimmune systemic disease.