A case report of isolated primary herpes-simplex virus neuroretinitis in an immunocompetent adult.

BACKGROUND: Herpes simplex virus (specifically HSV-1 and HSV-2) are greatly prevalent viruses that can cause conjunctivitis, keratitis and other rarer ocular disorders such as acute retinal necrosis syndrome or neuroretinitis. We report a case of an isolated unilateral neuroretinitis with primary HSV infection in an immunocompetent adult without other related clinical features. CASE PRESENTATION: A 60-year-old immunocompetent woman presented with sudden painless central vision loss in her left eye (best corrected visual acuity was 20/200) showing optic disc edema, submacular fluid and a delayed development of a macular star. The macular optical coherence tomography (OCT) showed a serous retinal detachment. Arterial hypertension or exposure to ionizing radiation were ruled out and the microbiological blood test battery was only positive for immunoglobulin M (IgM) for HSV-1 which allowed etiological treatment with oral valacyclovir. Complete resolution and good visual results were found within 3 months. CONCLUSIONS: The present case of isolated neuroretinitis as a primary HSV infection in an immunocompetent patient was resolved with good functional results after valacyclovir treatment. Presence of HSV IgM in absence of other laboratory results could be enough evidence to start HSV treatment in immunocompetent patients with a macular star, as an isolated lesion, after ruling out other non-infectious causes, such as arterial hypertension or exposure to ionizing radiation. Rare infectious agents in immunocompetent patients must be considered in the differential diagnosis of neuroretinitis, even if there are no other typical symptoms or signs that could suggest the disease.

New insights into the genetic component of non-infectious uveitis through an Immunochip strategy.

BACKGROUND: Large-scale genetic studies have reported several loci associated with specific disorders involving uveitis. Our aim was to identify genetic risk factors that might predispose to uveitis per se, independent of the clinical diagnosis, by performing a dense genotyping of immune-related loci. METHODS: 613 cases and 3693 unaffected controls from three European case/control sets were genotyped using the Immunochip array. Only patients with non-infectious non-anterior uveitis and without systemic features were selected. To perform a more comprehensive analysis of the human leucocyte antigen (HLA) region, SNPs, classical alleles and polymorphic amino acid variants were obtained via imputation. A meta-analysis combining the three case/control sets was conducted by the inverse variance method. RESULTS: The highest peak belonged to the HLA region. A more detailed analysis of this signal evidenced a strong association between the classical allele HLA-A*2902 and birdshot chorioretinopathy (p=3.21E-35, OR=50.95). An omnibus test yielded HLA-A 62 and 63 as relevant amino acid positions for this disease. In patients with intermediate and posterior uveitis, the strongest associations belonged to the rs7197 polymorphism, within HLA-DRA (p=2.07E-11, OR=1.99), and the HLA-DR15 haplotype (DRB1*1501: p=1.16E-10, OR=2.08; DQA1*0102: p=4.37E-09, OR=1.77; DQB1*0602: p=7.26E-10, OR=2.02). Outside the HLA region, the MAP4K4/IL1R2 locus reached statistical significance (rs7608679: p=8.38E-07, OR=1.42). Suggestive associations were found at five other loci. CONCLUSIONS: We have further interrogated the association between the HLA region and non-infectious non-anterior uveitis. In addition, we have identified a new non-HLA susceptibility factor and proposed additional risk loci with putative roles in this complex condition.

Vogt-Koyanagi-Harada disease in Spain.

PURPOSE: To describe the clinical and epidemiological characteristics of patients with Vogt-Koyanagi-Harada (VKH) disease in Spain. METHODS: This was a retrospective multicenter analysis of data from VKH patients followed for at least 6 months. The data collected were related to demographics, clinical manifestations, treatments, and complications. RESULTS: Participants were 112 patients (224 eyes), from 13 tertiary referral centers, of mean age 37.5 ± 14.7 years; 83.9% were women. Ethnicities were 61.6% Caucasian and 30.4% Hispanic. The disease was classified as complete in 16.1%, incomplete in 55.4%, and probable in 28.6%. When seen for the first time, the clinical course was acute in 69.6%, recurrent chronic in 15.2%, and chronic in 14.3%. The most frequent treatment was corticosteroids (acute stage 42.2%, maintenance stage 55.6%). The most common complications were cataract (41.1%) and ocular hypertension (16.1%). In most eyes, visual acuity was improved (96.7%) or remained stable at the end of follow up. CONCLUSION: VKH in Spain mostly affects women and presents as incomplete acute stage disease. Visual prognosis is good. Cataract and glaucoma are the two most frequent complications.

Takayasu's retinopathy.

We report the case of a 63-year-old woman with Takayasu's arteritis who experienced progressive visual loss in her right eye (RE) over several months. Visual acuity was 0.4 in the RE, which showed marked retinal arteriovenous dilation and highly irregular arteriolar calibre. She had no light perception in the left eye, which showed diffuse atrophy of the retinal pigment epithelium and prepapillary fibrovascular proliferation. Fluorescein angiography revealed delayed and slow retinal and choroidal circulation and areas of peripheral ischemia. A diagnosis of Takayasu's retinopathy was made and the non-perfused areas were treated with laser photocoagulation. Takayasu's disease can result in chronic ocular ischemia. Angiographic examination is particularly important in this context and may provide additional findings which affect staging and treatment of the disease. The role of the ophthalmologist includes laser photocoagulation, monitoring for complications and timely referral for vascular surgery.

Specific association of IL17A genetic variants with panuveitis.

BACKGROUND/AIMS: A pathogenic role of Th17 cells in uveitis has become clear in recent years. Therefore, in the present study, we aimed to evaluate the possible influence of the IL17A locus on susceptibility to non-anterior uveitis and its main clinical subgroups. METHODS: Five IL17A polymorphisms (rs4711998, rs8193036, rs3819024, rs2275913 and rs7747909), selected by tagging, were genotyped using TaqMan assays in 353 Spanish patients with non-anterior uveitis and 1851 ethnically matched controls. RESULTS: The case/control analysis yielded a consistent association between two of the analysed genetic variants, rs8193036 and rs2275913, and the presence of panuveitis under a dominant model (pFDR=2.86E-03, OR=2.26, 95% CI 1.42 to 3.59 and pFDR=0.033, OR=1.83, 95% CI 1.13 to 2.97, respectively). Subsequently, a specific association of both polymorphisms with the diffuse form of the disease was evident in the subphenotype analysis when considering this same genetic model (panuveitis vs posterior and intermediate uveitis: rs8193036, p=0.020; rs2275913, p=0.038). Independent effects of rs8193036 and rs2275913 were observed by conditional regression analysis. CONCLUSIONS: Polymorphisms within the IL17A locus show a novel association with panuveitis. Our data agree with the elevated levels of this cytokine that are found in patients with uveitis, supporting a crucial role of Th17 cells in this pathology. SUBTITLE: Our results clearly evidenced the role of IL17A as a novel genetic risk factor for panuveitis, thus suggesting the implication of Th17 cells in the extensive inflammation of the uveal tract that occurs in this subtype of uveitis.

Long-term efficacy and safety of infliximab in the treatment of Behçet's disease.

PURPOSE: To assess the long-term efficacy and safety of infliximab therapy for the treatment of Behçet's disease patients with ocular involvement who failed to respond or did not tolerate conventional treatment. METHODS: Retrospective study of 12 patients treated with infliximab at a starting dose of 5 mg/kg. RESULTS: Infliximab was infused during a mean of 31.43 months. The mean follow-up period was 35.77 months (range: 6-94). All patients achieved remission, 7 of whom did not need any adjuvant immunosuppressive therapy and 9 of whom were able to discontinue systemic corticosteroids. Visual acuity remained stable or improved in 20/21 eyes. Ten patients did not report any side effect of the medication or those were mild and tolerable. We observed two major adverse events requiring withdrawal of infliximab. CONCLUSIONS: Infliximab therapy is an effective biologic agent for the treatment of ocular inflammation in Behçet's disease unresponsive to the standard immunosuppressive therapy.

Treatment of proliferative diabetic retinopathy using viscosurgery with vital dye.

Viscosurgery is a surgical procedure based on the injection of viscoelastic material between the retinal surface and the fibrovascular tissue in proliferative diabetic retinopathy (PDR), with the aim of separating these structures to facilitate their dissection. We describe a modified viscosurgical technique in which the viscoelastic material is combined with a vital dye (trypan blue), which allows us to determine the degree of adhesion between the structures. Viscosurgery with vital dye has advantages compared with conventional viscosurgery and constitutes a useful surgical approach in complicated cases of severe PDR, enhancing efficiency and safety in the dissection of fibrovascular proliferations and minimizing iatrogenic damage to the underlying retina. We used trypan blue viscosurgery in 8 eyes of 8 patients with severe PDR; in all cases, a satisfactory outcome was obtained without evidence of any complications.