Treatment and prognosis of Vogt–Koyanagi–Harada disease: real-life experience in long-term follow-up

Abstract: Background: Vogt–Koyanagi–Harada (VKH) disease is a form of uveitis that is rare in Western countries. The aim of this study was to report on the long-term real-life treatment and prognosis of VKH in Italy. Methods: The clinical features, complications, and final visual acuity were retrospectively evaluated in 38 patients with VKH (mean follow-up: 120 months) globally, according to oral or intravenous corticosteroid treatment at onset and subsequent immunosuppressive therapy. Results: The mean final visual acuity was 0.13 ± 0.4 logMAR, which was a significant increase from the baseline (p < 0.0001). The patients who received intravenous rather than oral corticosteroids relapsed less (p = 0.026), with fewer relapses/patient/month of follow-up (p < 0.0001), and showed less frequent sunset glow fundus (33.3% versus 55%) and more relapse-free cases after induction therapy (p = 0.007). Delayed immunosuppressive therapy (median: 180 days from the onset of symptoms) reduced the rate of sunset glow fundus. The onset of sunset glow fundus was associated with a worse final visual acuity (p = 0.006). Conclusion: The long-term prognosis of VKH is quite good. Intravenous corticosteroids given at the onset of VKH are more effective than oral corticosteroids. Even if it is not given immediately after symptoms onset, immunosuppressive therapy is able to reduce the incidence of sunset glow fundus and to improve the final visual prognosis

Management of spontaneous crystalline lens luxation in a patient diagnosed with Takayasu's disease

Takayasu's disease is a chronic granulomatous arteriopathy that affects large vessels and their major branches. Nonspecific symptoms characterize the early phase, whereas findings of arterial occlusion and aneurysmal formation become manifest later. Ocular signs typically refer to retinal vascular involvement, as Takayasu arteritis or hypertensive retinopathy. We report a case of a 63-year-old woman suffering from Takayasu arteritis that complained of sudden onset of blurred vision in her left eye due to crystalline lens luxation in the vitreous cavity. The patient's past medical history was unremarkable for trauma, personal or familiar collagenopathies. Prompt surgical management was performed and the patient reached 0 LogMAR seven days after surgery. Our case illustrates the concomitant occurrence, never reported before, of two rare conditions in the same patient, namely, Takayasu arteritis and spontaneous lens dislocation. Further research and future knowledge are needed to explain whether Takayasu arteritis could obliquely injure zonular or fibrillar structures and whether these features may be possibly related

A branch retinal artery occlusion in a patient with Gaucher disease

Gaucher disease (GD) is a rare familial autosomal recessive disorder of lipid metabolism, resulting in an accumulation of abnormal glucocerebrosides in the reticulo-endothelial system. Patients with GD may present with hepatosplenomegaly, anemia, thrombocytopenia, and destructive bone disease. An enzyme replacement therapy with intravenous infusions of glycosylceramidase has been successfully proposed for treating the visceralmanifestations. Gaucher disease can be divided into three subtypes: non-neuronopathic (type 1) which is the most common, acute neuronopathic (type 2), and subacute neuronopathic (type 3). Several studies have reported ocular manifestations such as strabismus, conjunctival pterygia, corneal opacities, vitreous opacities and retinal involvement. To our knowledge, this is the first reported case of Gaucher disease complicated by branch retinal artery occlusion

Prognosis of juvenile rheumatoid arthritis-associated uveitis

PURPOSE. To evaluate the clinical characteristics and the visual prognosis of uveitis in juvenile rheumatoid arthritis (JRA). METHODS. The authors examined 63 patients with uveitis and JRA observed from January 1985 to December 2000. The following characteristics of each patient were considered: age at first visit, age at onset of uveitis and arthritis, sex, laterality and localization of uveitis, ocular complications, antinuclear antibody (ANA) and human leukocyte antigen (HLA) DR11 positivity, and follow-up. A retrospective study on mid-time visual outcome and ocular complications was performed on 42 patients with more than 12 months of follow-up. RESULTS. A total of 76.2% of the patients were female, with a mean age of 8.1 years. Chronic anterior uveitis was bilateral in 77.8% of the cases and unilateral in 22.2%. Arthritis was oligoarticular at onset in 87.3% of cases, and polyarticular in 12.7%. Mean age at arthritis onset was 4.5 years and mean age at uveitis onset was 5.4 years. ANA were positive in 92% of cases and HLA DR11 was present in 36 of the 43 patients tested (83.7%). Among the 42 patients with more than 12 months of follow-up, ocular complications occurred in 90.5% of cases and the most frequent were cataract (64.4% of eyes) and band keratopathy (59.2% of eyes). Secondary glaucoma (25% of eyes) was associated with the worst visual prognosis. A total of 64.5% of eyes maintained a visual acuity between 20/33 and 20/20 at the end of the follow-up. CONCLUSIONS. Visual prognosis of uveitis associated with JRA is improving, owing to earlier diagnosis and intensive treatment. Ocular complications occurred frequently in patients with uveitis and JRA but they did not seem to seriously affect the final visual outcome. The authors did not observe any correlation between prognosis and sex, age at the onset of uveitis or arthritis, pattern of arthritis, or positivity for ANA or HLA DR11. In a percentage of cases, uveitis may develop before arthritis or years after the onset of arthritis; therefore, continuous ophthalmologic examinations are needed in young people with JRA

Fuchs’ Heterochromic Iridocyclitis in an Italian Tertiary Referral Centre: Epidemiology, Clinical Features, and Prognosis

Purpose. To study epidemiology, clinical findings and visual prognosis of patients with Fuchs’ Heterochromic Iridocyclitis (FHI). Methods. A retrospective analysis was performed on 158 patients with FHI. Thirty-five patients were observed only once; the remaining 123 had a mean follow-up of 30.7 months (50 of them had a mean follow-up of 63.5 months) and in those we assessed complications, medical and surgical treatment, and long-term visual prognosis. Results. Average age at uveitis diagnosis was 27.2 years and 18.3% of patients were children. Blurred vision (54.5%) and floaters (40.5%) were the most frequent presenting symptoms. Small to medium-sized keratic precipitates (95.6%), iris atrophy (86.8%), and vitreous opacities (91.2%) were the most common signs; the prevalence of cataract and IOP increase was 63.5% and 20.1%, respectively, and their incidence was 0.1 and 0.06 eye/year. Significant risk factor for visual loss was IOP increase at presentation (p=0.02). At final examination 98% of the eye had a visual acuity ≥ 0.6, and topical (p<0.001) and systemic (p<0.001) corticosteroids therapy were used less frequently than before referral. Conclusions. FHI has a good visual prognosis, despite the significant incidence of cataract and glaucoma. A correct and prompt diagnosis might avoid unnecessary therapies and provide excellent visual outcomes