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Byline: S. Rathinam, Ilknur. Tugal-Tutkun, Mamta. Agarwal, Vedhanayaki. Rajesh, Merih. Egriparmak, Gazal. Patnaik Uveitis is a complex disorder including both infectious and non-infectious etiologies. Clinical diagnosis is a challenge because [...]

Objectives: To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF). Methods: Retrospective collaborative case series using invitation by email to uveitis specialists around the Mediterranean basin. This series was combined with a literature review. Exclusion criteria included infectious diseases, Behçet’s disease or other autoimmune diseases. Results: A total of 16 patients (21 eyes) had FMF and retinal vasculitis (FBA 11 patients, mild retinal vasculitis 5 patients). The mean age at onset of vasculitis was 29.5 ± 13.4 (range 9–62) with a female to male ratio of 9 to 7. In 19 eyes treated with various forms of corticosteroid and/or immunosuppression, the mean initial spectacle-corrected visual acuity improved from 6/194 to 6/10.5 at the last mean follow-up of 29.0 ± 34.9 months (p< 0.001). The most common FEVR mutations were M680I and M694V. In addition, retinal vascular occlusions included one case of central retinal artery occlusion and one case of branch retinal artery occlusion. Conclusion: FBA and milder forms of retinal vasculitis are associated with FMF. Therapy involves an increase in colchicine dosage in early cases, a long period of oral corticosteroid, intravitreal dexamethasone implant or periocular corticosteroid in select cases, and combination therapy with systemic immunosuppression in severe cases. FMF needs to be included in the differential diagnosis of retinal vasculitis.

Vogt–Koyanagi–Harada (VKH) disease is a primary autoimmune stromal choroiditis. This review aimed to provide a novel perspective of the disease. We took into account recent developments in the understanding of the disease and crucial progress in investigational modalities of the choroid, which has led to new, simpler diagnostic criteria. We analysed recent novel notions in the literature and new diagnostic tools for VKH. We identified the following updates for VKH disease: (1) A crucial differentiation between the acute initial-onset and the chronic forms of the disease; (2) the integration of new, precise imaging methods to assess choroidal inflammation; (3) the promotion of simplified, more reliable diagnostic criteria for acute initial-onset of the disease, based on the sine qua nonpresence of diffuse choroiditis, detected with indocyanine green angiography (ICGA) and/or Enhanced Depth Imaging OCT (EDI-OCT); and (4) treatment optimisation through early, vigorous, sustained corticosteroid and nonsteroidal immunosuppression, as the first line of treatment for initial-onset VKH disease, and monitoring subclinical choroidal inflammation during follow-ups. Several studies have shown that most patients could discontinue treatment without an inflammation relapse. ICGA and EDI-OCT represented the methods of choice for precisely monitoring disease evolution. Simplified, precise, new diagnostic criteria allow early diagnosis of VKH. In VKH disease, inflammation exclusively originates in the choroidal stroma. Therefore, in many cases, early, sustained treatment, with dual corticosteroid and nonsteroidal immunosuppressive therapy can result in full “healing”, which obviates chronic, uncontrolled, subclinical choroidal inflammation.

The aim of this review was to identify the imaging methods at our disposal to optimally manage posterior uveitis at the present time. The focus was put on methods that have become available since the 1990s, some 30 years after fluorescein angiography had revolutionized imaging of posterior uveitis in particular imaging of the retinal vascular structures in the 1960s. We have focussed our review on precise imaging methods that have been standardized and validated and can be used universally thanks to commercially produced and available instruments for the diagnosis and follow-up of posterior uveitis. The first part of this imaging review will deal with noninvasive imaging methods, focusing on fundus autofluorescence and optical coherence tomography as well as recent developments in imaging of the posterior segment.

AimsTo establish expert recommendations for the management of ocular sarcoidosis (OS).MethodsA question-based survey on the management of OS was circulated to international uveitis experts (members of the International Uveitis Study Group and the International Ocular Inflammation Society) electronically. Subsequently, a consensus workshop was conducted at the 7th International Workshop on Ocular Sarcoidosis (IWOS) in June 2019 in Sapporo, Japan as part of the Global Ocular Inflammation Workshops. Statements on the management of OS that were supported by a two-thirds majority of 10 international panel members of the workshop, after discussion and voting, were taken as consensus agreement.ResultsA total of 98 participants from 29 countries responded to the questionnaire survey. The subsequent consensus workshop established recommendations for the management of OS in five sections. The first section concerned evaluation and monitoring of inflammation. The second, third and fourth sections described ocular manifestations that were indications for treatment, and the management of anterior uveitis, intermediate uveitis and posterior uveitis. In the fifth section, the use of systemic corticosteroids and systemic immunosuppressive drugs were detailed.ConclusionsRecommendations for management of OS were formulated through an IWOS consensus workshop.

The aim of this review was to identify the imaging methods at our disposal to optimally manage posterior uveitis at the present time. The focus was put on methods that have become available since the 1990s, some 30 years after fluorescein angiography had revolutionised imaging of posterior uveitis in particular imaging of the retinal vascular structures in the 1960s. We have focussed our review on precise imaging methods that have been standardised and validated and can be used universally thanks to commercially produced and available instruments for the diagnosis and follow-up of posterior uveitis. The second part of this imaging review will deal with invasive imaging methods and in particular ocular angiography.

ABSTRACTIntroduction: Behçet’s disease uveitis (BDU) is a potentially blinding disorder. Systemic treatment with disease-modifying anti-rheumatic drugs (DMARDs) is mandatory in patients with intraocular inflammation involving the posterior segment of the eye.Areas covered: This article discusses existing systemic treatment with corticosteroids and conventional and biologic DMARDs as well as adjunctive local therapy in BDU. An overview is provided for a wide range of biologic DMARDs that have shown promise or investigated in clinical trials. Most recently introduced biologic DMARDs and targeted synthetic DMARDs are also reviewed for their potential in the treatment of BDU.Expert opinion: The prognosis of patients with BDU has remarkably improved after the introduction of biologic DMARDs. An expanding therapeutic armamentarium will allow treatment of most refractory cases. The ultimate goal is to provide drug-free remission with preservation of 20/20 vision.

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Byline: Samuray. Tuncer, Ilknur. Tugal-Tutkun Choroidal nevi are the most common benign pigmented lesions of the fundus. Choroidal neovascularization is a rare complication of choroidal nevi. We report herein a [...]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ajo.2009.04.007 Byline: Ilknur Tugal-Tutkun, Esra Guney-Tefekli, Fulya Kamaci-Duman, Isik Corum Abstract: To describe Turkish patients with Fuchs uveitis syndrome (FUS). Author Affiliation: Istanbul University, Istanbul Faculty of Medicine, Department of Ophthalmology, Istanbul, Turkey Article History: Accepted 8 April 2009 Article Note: (footnote) See accompanying Editorial on page 479.

We analyzed 1,900 Turkish Behçet's disease cases and 1,779 controls genotyped with the Immunochip. The most significantly associated SNP was rs1050502, a tag SNP for HLA-B*51. In the Turkish discovery set, we identified three new risk loci, IL1A–IL1B, IRF8, and CEBPB–PTPN1, with genome-wide significance (P < 5 × 10−8) by direct genotyping and ADO–EGR2 by imputation. We replicated the ADO–EGR2, IRF8, and CEBPB–PTPN1 loci by genotyping 969 Iranian cases and 826 controls. Imputed data in 608 Japanese cases and 737 controls further replicated ADO–EGR2 and IRF8, and meta-analysis additionally identified RIPK2 and LACC1. The disease-associated allele of rs4402765, the lead marker at IL1A–IL1B, was associated with both decreased IL-1α and increased IL-1β production. ABO non-secretor genotypes for two ancestry-specific FUT2 SNPs showed strong disease association (P = 5.89 × 10−15). Our findings extend the list of susceptibility genes shared with Crohn's disease and leprosy and implicate mucosal factors and the innate immune response to microbial exposure in Behçet's disease susceptibility.

IMPORTANCE: The best treatment option for primary vitreoretinal lymphoma (PVRL) without signs of central nervous system lymphoma (CNSL) involvement determined on magnetic resonance imaging or in cerebrospinal fluid is unknown. OBJECTIVE: To evaluate the outcomes of treatment regimens used for PVRL in the prevention of subsequent CNSL. DESIGN, SETTING, AND PARTICIPANTS: A retrospective cohort study was conducted at 17 referral ophthalmologic centers in Europe. We reviewed clinical, laboratory, and imaging data on 78 patients with PVRL who did not have CNSL on presentation between January 1, 1991, and December 31, 2012, with a focus on the incidence of CNS manifestations during the follow-up period. INTERVENTIONS: The term extensive treatment was used for various combinations of systemic and intrathecal chemotherapy, whole-brain radiotherapy, and peripheral blood stem cell transplantation. Therapy to prevent CNSL included ocular radiotherapy and/or ocular chemotherapy (group A, 31 patients), extensive systemic treatment (group B, 21 patients), and a combination of ocular and extensive treatment (group C, 23 patients); 3 patients did not receive treatment. A total of 40 patients received systemic chemotherapy. MAIN OUTCOMES AND MEASURES: Development of CNSL following the diagnosis of PVRL relative to the use or nonuse of systemic chemotherapy and other treatment regimens. RESULTS: Overall, CNSL developed in 28 of 78 patients (36%) at a median follow-up of 49 months. Specifically, CNSL developed in 10 of 31 (32%) in group A, 9 of 21 (43%) in group B, and 9 of 23 (39%) in group C. The 5-year cumulative survival rate was lower in patients with CNSL (35% [95% CI, 50% to 86%]) than in patients without CNSL (68% [95% CI, 19% to 51%]; P = .003) and was similar among all treatment groups (P = .10). Adverse systemic effects occurred in 9 of 40 (23%) patients receiving systemic chemotherapy; the most common of these effects was acute renal failure. CONCLUSIONS AND RELEVANCE: In the present series of patients with isolated PVRL, the use of systemic chemotherapy was not proven to prevent CNSL and was associated with more severe adverse effects compared with local treatment.

OBJECTIVE To describe a series of patients with bilateral acute iris transillumination, pigment dispersion, and sphincter paralysis. METHODS We reviewed the medical records and clinical photographs of 26 patients seen at 5 centers in Turkey and Belgium between March 16, 2006, and July 6, 2010. Observation procedures included clinical examination, anterior segment color photography, gonioscopy, laser flare photometry, and pupillometry. RESULTS All 26 patients (20 women and 6 men; mean [SD] age, 43.2 [10.5] years) had bilateral involvement. Twenty-three patients (88%) had acute-onset disease with severe photophobia and red eyes. Nineteen patients (73%) had a preceding flulike illness and used systemic antibiotics, including moxifloxacin. Diagnostic laboratory workup was unremarkable. There was pigment discharge into the anterior chamber, and flare was elevated in the absence of inflammatory cells. Most patients had severe diffuse transillumination of the iris and mydriatic distorted pupils. Pupillometry revealed a compromised reaction to light. The most serious complication was an intractable early rise in intraocular pressure. Gonioscopy revealed heavy pigment deposition in the trabecular meshwork. Although symptoms were relieved promptly by application of topical corticosteroid, the median duration of pigment dispersion was 5.25 months. CONCLUSIONS Bilateral acute iris transillumination with pigment dispersion and persistent mydriasis is a new clinical entity that is not an ocular adverse effect of oral moxifloxacin treatment, as previously suggested. The etiopathogenesis of this entity remains to be elucidated.

Purpose TO investigate the inflammation of the anterior chamber after intravitreal bevacizumab injection in different ocular exudative diseases.Methods The study included 76 eyes from 62 consecutive patients with different ocular exudative diseases. The patients were divided into the 3 following groups: group 1 (nonproliferative diabetic retinopathy), group 2 (choroidal neovascularization secondary to age-related macular degeneration), and group 3 (macular edema with branch or central retinal vein occlusion). The study also included 32 age-matched control patients. Inflammation of the anterior chamber was examined with flare-cell photometry before and after an intravitreal injection of 1.25 mg of bevacizumab.Results There were no statistically significant differences between the measurements at baseline and postoperative day 1, 3, 7, or 30 in any of the groups (p>0.05).Conclusions The extent of inflammation in the anterior chamber did not change after intravitreal bevacizumab injection in patients with nonproliferative diabetic retinopathy, choroidal neovascularization secondary to age-related macular degeneration, or macular edema due to branch or central vein occlusion.

Abstract: Purpose: To report on the results of interferon-α 2a (IFNα) treatment in patients with Behçet uveitis unresponsive to conventional immunosuppressive therapy.Methods: We retrospectively analyzed the medical records of 44 patients who had been treated with IFNα between September 2001 and May 2005. The initial dose of IFNα was 6 MU/day in 37 patients (84.1%) and 3 MU/day in 7 patients (15.9%), and was gradually tapered after ocular inflammation was suppressed. Immunosuppressive agents were discontinued. Oral corticosteroids were discontinued or maintained at a dosage of less than 10 mg prednisone equivalent per day. Main outcome measures were recurrence of posterior or panuveitis attacks and changes in visual acuity.Results: Sixteen patients (36.4%) remained relapse free during treatment, whereas 28 (63.6%) patients had recurrent uveitis attacks. Four of these were considered treatment failures and were switched to other treatments. In the remaining 40 patients, the mean duration of treatment was 12.4±10.8 months (range 3–45 months). In 9 of 40 patients (22.5%) treatment could be discontinued 22.2±13.4 months after therapy, and 8 (20%) of these patients had sustained remission for up to 24 months. Three patients (7.5%) were switched to other therapies because of side effects. The frequency of uveitis attacks per 6 months was reduced from 1.6±1.2 to 0.8±0.9 in 26 patients who had a minimum follow-up of 6 months before and after IFNα therapy (p<0.05). There was a significant improvement in visual acuity and this was preserved throughout follow-up in 38 (95%) of 40 patients.Conclusions: A partial or complete response was obtained with IFNα therapy in 91% of Turkish patients with Behçet uveitis refractory to conventional immunosuppressive treatment. Our results suggest that there may be differences in therapeutic efficacy and side-effect profile of IFNα in different patient populations. Comparative studies are needed to investigate this hypothesis.

To report on five cases of unusual bilateral stromal depigmentation of the iris and pigment dispersion masquerading as uveitis.We describe the clinical features of five consecutive patients who presented with acute depigmentation of the iris stroma between June and October 2003.Four patients were female, one was male. Age at presentation ranged from 15 to 25 years. Presenting symptoms were sudden-onset ocular discomfort and red eye in four patients and change of eye color in one patient. All patients had bilateral involvement, with a symmetrical diffuse depigmentation of the iris stroma in three cases and patchy areas of iris depigmentation in two. Other common features were mild ciliary injection (seven eyes), Krukenberg spindle (seven eyes), circulating pigment in the anterior chamber (eight eyes), and heavy pigment deposition in the angle (ten eyes). No eyes had iris transillumination defects, inflammatory keratic precipitates or inflammatory cells in the anterior chamber. Systemic laboratory work-up was unrewarding in all cases, and PCR analysis of the aqueous humor for HSV1 and 2 was negative in one patient. Four patients were treated with a short course of topical corticosteroids and three with oral acyclovir. One patient was lost to follow-up. The remaining four patients were followed up for 6–19 months with a stable clinical picture.In contrast to pigment dispersion syndrome, pigment seemed to be released from iris stroma in the five cases described here. Although patchy depigmentation of the iris resembled the lesions seen in herpetic iridocyclitis in two of the patients, symmetrical bilateral involvement and lack of intraocular inflammation were the differentiating features. The patients described here could represent a new entity or an unusual presentation of herpetic eye disease.

To investigate the mechanisms involved in the development of neovascularization of the optic disc (NVD) in Behçet's disease and to evaluate the effects of medical and laser treatments.This is a retrospective study of 26 consecutive Behçet's patients (38 eyes) with NVD who presented between 1990 and 2004 at our university hospital. Information on age at presentation, sex, disease duration, laterality of NVD, ocular findings, fluorescein angiographic findings, visual acuity, medical treatment, laser photocoagulation, surgical procedures, and the follow-up period was collected. Eyes with diffuse capillary leakage on fluorescein angiography were defined as having inflammation-induced NVD, and eyes with extensive retinal capillary nonperfusion were defined as having ischemia-induced NVD.Eighteen patients were male, and eight were female. Mean age at presentation was 25.4 ± 4.9 years. Median disease duration was 5.5 months. Median follow-up was 24 months. Twelve patients had bilateral NVD; 14 had unilateral NVD. Inflammation-induced NVD was seen in 87% of the eyes, and ischemia-induced NVD in 13%. Initial treatment with high-dose corticosteroids combined with conventional immunosuppressive agents was effective in 45% of the eyes with inflammation-induced NVD. Retinal laser photocoagulation was effective in three of five eyes with ischemia-induced NVD. Treatment with interferon α-2a resulted in resolution of NVD in all seven patients who received this agent for inflammation- or ischemia-induced persistent NVD. Final visual acuity was less than 0.1 in 24% of the eyes.Inflammatory mechanisms seem to predominate in the pathogenesis of NVD in Behçet's disease. The results of this study suggest that the response to intensive anti-inflammatory and conventional immunosuppressive treatment is not satisfactory; retinal laser photocoagulation may be ineffective even in eyes with retinal ischemia, but interferon α-2a seems to be effective for the treatment of Behçet's patients with NVD. Jpn J Ophthalmol2006;50:256–265 © Japanese Ophthalmological Society 2006

AbstractCytomegalovirus retinitis (CMVR) is a rare complication of systemic immunosuppressive therapy in patients with rheumatic disorders. We describe a 44-year-old man with Wegener’s granulomatosis who was treated with cyclophosphamide and methylprednisolone and who subsequently developed bilateral CMVR. In spite of discontinuation of immunosuppressive therapy and administration of systemic ganciclovir, retinitis recurred and maintenance therapy was needed. In conclusion, increased awareness of this potentially serious complication of immunosuppressive therapy is critical. If immune recovery is slow after discontinuation of immunosuppressive agents, prolonged antiviral therapy is required in order to prevent recurrences of CMVR.Copyright © 2000 S. Karger AG, Basel

Purpose:Even though conjunctiva is not primarily involved in patients with uveitis due to Behçet disease, it may reflect the immunopathologic process when inflammation is induced by biopsy of conjunctiva, a phenomenon similar to the induced inflammation at skin pathergy sites.

Background:Although uveitis is relatively uncommon in children, its diagnosis and management present a distinct clinical challenge for the physician. An improved knowledge of disease patterns and associated morbidity will help in the care of children with uveitis.