Chronic Recurrent Vogt-Koyanagi-Harada Disease and Development of 'Sunset Glow Fundus' Predict Worse Retinal Sensitivity.

PURPOSE: To investigate prognostic factors for retinal sensitivity assessed by microperimetry in patients with Vogt-Koyanagi-Harada (VKH) disease. METHODS: In total, 34 patients with initial-onset acute disease and 19 patients with chronic recurrent disease were retrospectively evaluated. RESULTS: The mean follow-up period was 40.4 ± 40.5 months. Sensitivity was significantly worse in eyes with more severe anterior segment inflammation at presentation, as indicated by the presence of mutton-fat keratic precipitates, anterior chamber reaction ≥2+, and posterior synechiae. Chronic recurrent presentation, development of complications, and 'sunset glow fundus' were significantly associated with worse sensitivity. Using logistic regression analysis, better sensitivity was significantly associated with initial-onset acute presentation (odds ratio, OR = 6.9; 95% confidence interval, CI = 1.53-9.66). CONCLUSIONS: Chronic recurrent presentation and development of complications and 'sunset glow fundus' are associated with a worse sensitivity outcome.

Mycophenolate mofetil combined with systemic corticosteroids prevents progression to chronic recurrent inflammation and development of 'sunset glow fundus' in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease.

PURPOSE: To evaluate the effectiveness and safety of mycophenolate mofetil (MMF) as first-line therapy combined with systemic corticosteroids in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. METHODS: This prospective study included 38 patients (76 eyes). The main outcome measures were final visual acuity, corticosteroid-sparing effect, progression to chronic recurrent granulomatous uveitis and development of complications, particularly 'sunset glow fundus'. RESULTS: The mean follow-up period was 37.0 ± 29.3 (range 9-120 months). Visual acuity of 20/20 was achieved by 93.4% of the eyes. Corticosteroid-sparing effect was achieved in all patients. The mean interval between starting treatment and tapering to 10 mg or less daily was 3.8 ± 1.3 months (range 3-7 months). Twenty-two patients (57.9%) discontinued treatment without relapse of inflammation. The mean time observed off of treatment was 28.1 ± 19.6 months (range 1-60 months). None of the eyes progressed to chronic recurrent granulomatous uveitis. The ocular complications encountered were glaucoma in two eyes (2.6%) and cataract in five eyes (6.6%). None of the eyes developed 'sunset glow fundus', and none of the patients developed any systemic adverse events associated with the treatment. CONCLUSIONS: Use of MMF as first-line therapy combined with systemic corticosteroids in patients with initial-onset acute VKH disease prevents progression to chronic recurrent granulomatous inflammation and development of 'sunset glow fundus'.

Indocyanine green angiographic findings in initial-onset acute Vogt-Koyanagi-Harada disease.

PURPOSE: To study the features of Indocyanine green angiography (ICGA) in patients with initial-onset acute Vogt-Koyanagi-Harada (VKH) disease. METHODS: Retrospective cohort study of ICGA performed with the use of Heidelberg scanning laser ophthalmoscope on a consecutive series of patients with initial-onset acute VKH disease. The following signs were analysed: choroidal perfusion inhomogeneity, early hyperfluorescent stromal vessels, hypofluorescent dark dots (HDDs), fuzzy or lost pattern of large stromal choroidal vessels, disc hyperfluorescence, and, diffuse late choroidal hyperfluorescence. In addition, we looked for any new ICGA findings. RESULTS: Thirty-six patients (72 eyes) from a single academic institution were studied. The following findings were identified: HDDs in all eyes (100%), fuzzy or lost pattern of large stromal vessels in all eyes (100%), early hyperfluorescent stromal vessels were seen in 60 eyes (83%), diffuse late choroidal hyperfluorescence was present in 51 eyes (71%), choroidal perfusion inhomogeneity was seen in 44 eyes (61%), disc hyperfluorescence was seen in 25 cases (69%), and choroidal folds were present in only six eyes. New ICGA findings detected in this study were hypofluorescent patches corresponding to areas of exudative retinal detachment in 60 eyes (83%) and 'starry sky' pattern of late widespread punctate choroidal hyperfluorescence in 37 eyes (51%). CONCLUSIONS: The most prevalent ICGA findings were HDDs, fuzzy stromal vessels and early hyperfluorescent stromal vessels in patients with initial-onset acute VKH disease. Novel ICGA findings in this group of patients included hypofluorescent patches corresponding to areas of exudative retinal detachment, and late widespread punctate choroidal hyperfluorescence taking on a 'starry sky' pattern.

Incidence and Risk Factors for Developing Glaucoma Among Patients with Uveitis in a University-based Tertiary Referral Center in Riyadh, Saudi Arabia.

PURPOSE: To investigate the incidence and risk factors of secondary glaucoma among uveitis patients. METHODS: Retrospective review of medical records of 642 patients (1220 eyes). RESULTS: Glaucoma was diagnosed in 169 (13.9%) eyes and was most common in eyes with anterior uveitis (19.1%) (p < 0.001). HLA-B27-positive anterior uveitis (27.6%), Fuchs' uveitis (23.3%), juvenile idiopathic arthritis (23.1%), herpetic uveitis (20.3%), and Vogt-Koyanagi-Harada disease (16.3%) were the leading clinical entities associated with glaucoma (p < 0.001). Significant risk factors at presentation included worse visual acuity, elevated intraocular pressure, keratic precipitates, granulomatous inflammation, anterior chamber reaction≥ 2+, posterior synechiae, and cataract. Female gender and iris nodules significantly predicted the need for glaucoma surgery. Visual outcome was worse in eyes with glaucoma than in eyes without glaucoma. CONCLUSIONS: Incidence of glaucoma differed depending on anatomic and etiologic diagnoses of uveitis. There is a significant association between severity of inflammation at presentation and development of glaucoma.

Patterns of Uveitis in a University-based Tertiary Referral Center in Riyadh, Saudi Arabia.

PURPOSE: To investigate the referral patterns and diagnosis of uveitis in a university-based tertiary referral center in Riyadh, Saudi Arabia. METHODS: We retrospectively reviewed the medical records of 642 patients (1220 eyes). RESULTS: There were 295 (46%) males and 347 (54%) female patients, with a mean age of 36.4 ± 16.1 years at presentation. Panuveitis was most common (47.1%), followed by anterior uveitis (36.8%), posterior uveitis (10.7%), and intermediate uveitis (5.4%). Nongranulomatous (85.2%) and noninfectious (69.3%) were the most frequent types of uveitis. The most identifiable specific diagnoses were Vogt-Koyanagi-Harada (VKH) disease (19.6%), presumed tuberculous uveitis (PTU) (17.8%), Behçet disease (BD) (8.4%), and toxoplasmosis (6.9%). After a mean follow-up period of 31.3 ± 31.5 months, 73.5% of the eyes achieved visual acuity of 20/40 or better. CONCLUSIONS: The most common anatomic diagnosis was panuveitis. VKH disease, PTU, BD, and toxoplasmosis are the most frequently diagnosed entities.

Prognostic factors for clinical outcomes in patients with Vogt-Koyanagi-Harada disease treated with high-dose corticosteroids.

PURPOSE: To determine prognostic factors in patients with Vogt-Koyanagi-Harada (VKH) disease who were treated with high-dose corticosteroids. METHODS: Retrospective analysis of 87 patients (174 eyes). RESULTS: At presentation, there were 53 patients with initial-onset acute VKH disease and 34 patients with chronic recurrent VKH disease. Chronic recurrent presentation was significantly associated with more severe anterior segment inflammation at presentation as indicated by presence of mutton-fat keratic precipitates, anterior chamber reaction ≥2+, iris nodules and posterior synechiae (p < 0.001 for all comparisons), less exudative retinal detachment at presentation (p < 0.001), more complications during the follow-up period (p < 0.001) and a worse visual outcome (p < 0.001). The use of immunomodulatory therapy (cyclosporine and mycophenolate mofetil) as first-line therapy significantly reduced the development of complications in the whole study group (p = 0.006) and in initial-onset acute group (p = 0.024) and improved visual outcome in the whole study group (p = 0.004) and in chronic recurrent group (p = 0.024). In the whole study group, final visual acuity of 20/20 was significantly associated with good initial visual acuity of >20/200 [odds ratio = 4.25; 95% Confidence interval (CI) = 1.53-11.89] and age older than 16 years was significantly associated with the development of complications (odds ratio = 3.15; 95% CI = 1.04-9.48). CONCLUSIONS: Chronic recurrent VKH disease is significantly associated with more severe anterior segment inflammation and less exudative retinal detachment at presentation, more ocular complications and a worse visual outcome than initial-onset acute VKH disease. Use of immunomodulatory therapy significantly improved the clinical outcomes.

The outcomes of mycophenolate mofetil therapy combined with systemic corticosteroids in acute uveitis associated with Vogt-Koyanagi-Harada disease.

PURPOSE: To study the effectiveness of mycophenolate mofetil (MMF) as first-line therapy combined with systemic corticosteroids in acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. The outcomes in this group were compared with those of another group of patients with VKH disease who were treated with corticosteroid monotherapy or with delayed addition of immunomodulatory therapy. METHODS: This prospective study included 19 patients (38 eyes) diagnosed with acute uveitis associated with VKH disease. RESULTS: The mean follow-up period was 27.0 ± 11.1 months (range 16-54 months). Corticosteroid-sparing effect was achieved in all patients. The mean interval between starting treatment and tapering prednisone to 10 mg or less daily was 5.1 ± 1.2 months (range 3-7 months). Ten (53%) patients discontinued treatment without relapse of inflammation. The mean time observed of treatment was 17.3 ± 11.9 months (range 3-41.5 months). Visual acuity of 20/20 was achieved by 38% of the eyes in the corticosteroid group and by 74% in the corticosteroid + MMF group (p < 0.001). Recurrent inflammation of ≥3 times was reduced significantly (p = 0.0383) in the corticosteroid + MMF group (3%) as compared to corticosteroid group (18%). Development of all complications was significantly higher in the corticosteroid group (43%) compared with the corticosteroid + MMF group (8%) (p < 0.001). None of the eyes in the corticosteroid + MMF group developed 'sunset glow fundus'. CONCLUSIONS: Addition of MMF as first-line therapy to corticosteroids in patients with acute uveitis associated with VKH disease leads to significant reduction in recurrences of uveitis and development of late complications and significantly improves visual outcome.

Retinal functional changes measured by microperimetry after immunosuppressive therapy in patients with Vogt-Koyanagi-Harada disease.

PURPOSE: To evaluate the effect of immunosuppressive therapy on best-corrected visual acuity (BCVA), fixation stability, and central retinal sensitivity in patients with Vogt-Koyanagi-Harada (VKH) disease in the acute uveitic phase. Methods. In this prospective study, 14 patients (28 eyes) were evaluated. Best-corrected visual acuity and MP-1 microperimetric evaluation of retinal sensitivity in the central 12 degrees and fixation stability were assessed at baseline and at 1, 3, 6, 9, and 12 months after treatment. Results. At baseline, logarithm of the minimum angle of resolution (logMAR) BCVA, fixation stability, and mean retinal sensitivity levels were 0.685 ± 0.6 (Snellen equivalent, 20/100), 61.2% ± 29.0%, and 2.75 ± 3.8 dB, respectively. At 3 months, logMAR BCVA (0.11 ± 0.2, Snellen equivalent 20/25) and fixation stability (86.4% ± 13.4%) were almost maximum (p<0.001 for both comparisons), and thereafter remained almost unchanged. Mean retinal sensitivity continued to improve up to 12 months (12.0 ± 2.3 dB, p<0.001); however, it was still significantly decreased. There were significant correlations between logMAR BCVA and mean retinal sensitivity at all time points. The percentages of BCVA improvement were significantly higher than the percentages of mean retinal sensitivity improvement at all time points (p<0.001 for all comparisons). Conclusions. Compared with microperimetry, BCVA significantly underestimates macular dysfunction in VKH disease.