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Familial Mediterranean fever is an autoinflammatory multisystem disease, which most commonly affects patients from the Mediterranean basin. This review discusses the common polymorphisms in the MEFV gene as well as the role of pyrin in disease pathogenesis. Patients with familial Mediterranean fever typically develop peritonitis, pleuritis, arthritis, and fever. In addition, a number of authors have reported ophthalmic features. These case reports and series are further explored in this review. Colchicine has transformed the prognosis for patients with familial Mediterranean fever. The rationale for the use of colchicine, as well as the evidence for newer biologic agents is also covered. [ABSTRACT FROM AUTHOR]

Background: Treatment of ocular toxoplasmosis is aimed at stabilizing visual function and reducing recurrence rates. Methods: Small controlled studies indicate that available treatments do not affect visual outcome and recurrence rates, and no antibiotic in current use will kill bradyzoites. Results: Antiparasitic treatment is justified in center-involving lesions and in large aggressive lesions namely in South American patients. Antibiotic treatment is needed for disease in the immunosuppressed, and this needs to be systemic. There exists strong agreement that a monotherapy, using steroids, is contraindicated. Prophylactic antibiotics may reduce recurrence rates in endemic areas and immunosuppressed patients. Conclusion: An ideal therapeutic strategy includes the strain of parasite, localization of the lesion, and severity of the inflammatory response as a basis for therapeutic decision making. New treatments targeting aspects of the parasite s physiology are very promising. On a global scale, public health measures to prevent transmission from animals and to access potable water are required. [ABSTRACT FROM AUTHOR]

Since there is no pathognomonic clinical sign or laboratory test to distinguish Behçet disease from other uveitic entities, the diagnosis must be made based on characteristic ocular and systemic findings in the absence of evidence of other disease that can explain the findings. Ancillary tests, including ocular and brain imaging studies, are used to assess the severity of intraocular inflammation and systemic manifestations of Behçet disease, to identify latent infections and other medical conditions that might worsen with systemic treatment, and to monitor for adverse effects of drugs used. There are two diagnostic or classification criteria in general use by the uveitis community, one from Japan and one from an international group; both rely on a minimum number and/or combination of clinical findings to identify Behçet disease. Finally, several grading schemes have been proposed to assess severity of ocular disease and response to treatment. [ABSTRACT FROM AUTHOR]

Purpose: To review current evidence for the treatment of ocular toxoplasmosis (OT). Design: Narrative review and expert recommendations. Methods: Meta-analysis and selected original articles from the medical literature were reviewed critically. Expert recommendations were analyzed. Results: Numerous observational studies suggest a benefit of short-term antimicrobial therapy for toxoplasmic retinochoroiditis in immunocompetent patients, although its efficacy has not been proven in randomized clinical trials. A randomized clinical trial revealed that intermittent trimethoprim/sulfamethoxazole treatment could decrease the rate of recurrence in high-risk patients. Intravitreal injection of clindamycin and dexamethasone was an acceptable alternative to the classic treatment for OT in a randomized clinical trial. Conclusions: Opinions about therapy differ and controversy remains about its type, efficacy, and length. Intravitreal therapy may be promising for OT. A recent description of the presence of parasitemia in patients with active and inactive ocular toxoplasmosis raises new questions that need to be explored. [ABSTRACT FROM AUTHOR]

Purpose: To assess whether the double-pass technique can be employed to quantify the amount of light scattering in patients with uveitis. Methods: 56 eyes of 44 patients with intraocular inflammation were consecutively recruited from the uveitis clinic over 9 months. The degree of intraocular inflammation was recorded according to the Standardization of Uveitis Nomenclature criteria and the eyes were grouped as having anterior, intermediate, posterior or panuveitis. Objective scatter index (OSI) was assessed using a double-pass technique with the Optical Quality Analysis System II. Results: Twenty-four eyes had anterior uveitis, 9 eyes had intermediate uveitis, 10 eyes had posterior uveitis and 13 eyes had panuveitis. The OSI was significantly different between all 4 groups (p = 0.0005). The mean OSI was highest in eyes with anterior uveitis (2.6 ± 3.1) and lowest in posterior uveitis (1.9 ± 1.3). Anterior chamber cells significantly correlated with OSI (R2 = 0.8726, p = 0.007), unlike posterior chamber cells (R2 = 0.0189, p = 0.588) and flare (R2 = 0.0048, p = 0.471). Conclusion: Patients with anterior uveitis have more ocular scatter, and anterior chamber cells scatter more light. This pilot study opens new avenues for research in use of the double-pass technique to assess light scattering in uveitis. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

: PurposeTo determine the effectiveness of systemic antibiotic treatment for toxoplasmic retinochoroiditis.: Clinical relevanceToxoplasma retinochoroiditis is a significant cause of visual morbidity. Multiple different antibiotic regimens are used, but controversy about treatment effectiveness remains.: Literature reviewedSearches were conducted of Cochrane Controlled Trials Register, Medline (1966 onward), Embase (1980 onward), Dissertation Abstracts (1861 onward), Lilacs (1982 onward), and Pascal (1984 onward). Pharmaceutical companies were contacted for unpublished data. Any randomized controlled trials that compared antibiotics versus placebo in immunocompetent patients with toxoplasmic retinochoroiditis were retrieved. Primary outcome measures were long-term visual acuity and risk of recurrent retinochoroiditis. Secondary outcomes included duration and severity of acute symptoms, size of the lesion at end of follow-up, and adverse effects of treatment.: ResultsOnly 3 studies (total of 173 participants) were randomized controlled trials and hence met the inclusion criteria (level II). All 3 were methodologically poor, and 2 were carried out more than 35 years ago. None reported the effect on long-term visual outcome. We found no evidence for a beneficial effect on the duration and severity of signs of acute toxoplasmic retinochoroiditis (A,II). There was weak evidence for an effect of long-term treatment for chronic recurrent toxoplasmic retinochoroiditis on lesion recurrence. Treatment was associated with adverse effects.: ConclusionsThere is a lack of evidence to support routine antibiotic treatment for acute toxoplasmic retinochoroiditis. Placebo-controlled randomized trials of antibiotic treatment in patients presenting with acute or chronic toxoplasmic retinochoroiditis arising in any part of the retina are required. [Copyright &y& Elsevier]

Purpose: To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings. Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies. [ABSTRACT FROM AUTHOR]

This article presents the case of a 65-year-old man with chronic lymphocytic leukaemia (CLL) who had a fungal infection at unusual sites as a result of combination therapy with alemtuzumab and methyl prednisolone. It discusses the clinical history of the patient, the prevalence of fungal infections in CLL and the management of the patient.

Describes cases of young patients who developed central retinal vein occlusion (CRVO) following an episode of dehydration. Cause of venous thrombosis; Characteristics of the retinal venous circulation; State of acute hyperviscosity in patients who had severe dehydrational episode.

Purpose: We report two cases of endogenous meningococcal endophthalmitis in immunocompetent adults presenting with unilateral ocular disease and joint involvement.Methods: A retrospective review of two patients with endogenous endophthalmitis due to Neisseria meningitidis with associated arthropathy was conducted. The clinical presentation, investigations, treatment, and outcomes are presented.Results: Vitreous sampling confirmed Neisseria meningitidis via polymerase chain reaction (PCR) in both cases. PCR was positive from the knee aspirate of our second case. Both patients received topical steroids and topical, intravitreal and intravenous antibiotics. Despite this, visual outcomes were poor.Conclusions: This atypical combination of endogenous meningococcal endophthalmitis associated with meningococcal joint involvement could represent a novel pattern for the presentation of metastatic meningococcal endophthalmitis not previously described. We suggest joint aspirate PCR could be a useful adjunctive test to identify potential causative organisms in such cases where there is concurrent joint involvement in the absence of systemic features. [ABSTRACT FROM AUTHOR]

Aim: To analyze current understanding of the factors that contribute to raised intraocular pressure (IOP) in patients with uveitis. Methods: A pubmed literature review was carried out using words including “uveitic glaucoma”, “IOP AND uveitis”, “ocular hypertension AND uveitis”, “inflammation AND glaucoma”, “aqueous dynamics” AND “glaucoma/uveitis”. Results: Of the two studies looking at the aqueous dynamics in experimentally induced uveitis, both found aqueous flow decreased acutely, and one found that uveoscleral outflow increased. This is likely to reflect the types of uveitis that present acutely with hypotony. A study examining patients with Fuch’s heterochromic cyclitis found no difference in aqueous flow or uveoscleral outflow. No studies have examined aqueous dynamics in types of uveitis that present with acutely raised IOP. Levels of prostaglandins rise in acute uveitis, which has been shown to increase uveoscleral and trabecular outflow, without affecting aqueous flow. Studies have demonstrated that raised levels of trabecular protein reduce trabecular outflow. Steroid treatment, inflammatory cells, free radicals and enzymes are also likely to contribute to the development of raised pressure. When considering the impact of the pathogenesis of raised pressure in uveitis on its treatment, prostaglandins may provide good intraocular pressure control, but there are concerns regarding their theoretical ability to worsen the inflammatory response in uveitis. Studies have not conclusively proven this to be the case. Surgical success rates vary, but trabeculectomy plus an antimetabolite, deep sclerectomy plus an antimetabolite, and Ahmed valve surgery have been used. Conclusions: Uveitic glaucoma is caused by a number of different diseases, some of which present with acute hypotony, others with acutely raised IOP, and others which demonstrate an increase in IOP over time. Further studies should be carried out to examine the differing pathogenesis in these types of diseases, and to establish the best treatment options. [ABSTRACT FROM AUTHOR]

Retinal infection with Toxoplasma gondii is the most important cause of posterior uveitis, whereby prevalence and incidence of ocular symptoms after infection depend on socio-economic factors and the circulating parasite genotypes. Ocular toxoplasmosis is more common in South America, Central America, and the Caribbean and parts of tropical Africa as compared to Europe and Northern America, and is quite rare in China. Ocular disease in South America is more severe than in other continents due to the presence of extremely virulent genotypes of the parasite. Drinking untreated water is considered the major source of Toxoplasma infection in developing countries, whereas in the Western world the consumption of raw or undercooked meat (products) is the most important cause. Since acquired infection with T. gondii is currently a more important cause of ocular toxoplasmosis compared to congenital infection, prevention should be directed not only toward pregnant women but toward the general population. [ABSTRACT FROM AUTHOR]

Falls are an important health issue. They cause significant morbidity and mortality particularly in older people, and also have marked psychological effects on the individual. The literature focuses particularly on older adults, an age group in which both visual impairment and falls are more prevalent, as is the associated morbidity. In this review, we summarise the current literature and point to further studies which need to be undertaken. The consequences of falls are well recognised, and there has been considerable work into identifying risk factors. Changes in visual components such as visual field, acuity, contrast sensitivity and stereopsis all have a part and the co-existence of other sensory impairments certainly increases the risk of falls. However there remain considerable gaps in our knowledge of the relationship between visual loss and falls, for example in patients with diabetic eye disease. Furthermore, there is also conflicting data as to the importance of different visual components. Various interventions, such as programmed inter-disciplinary involvement, have shown promise, however these need further confirmation of their efficacy and cost effectiveness. An added confounder may be that an intervention (eg, cataract extraction) paradoxically affects an individual's future activity level and behaviour, thereby increasing the risk of falling. With an ageing population the importance of this topic is likely to increase, as will the potential benefits of optimising our assessment and management of these patients. [ABSTRACT FROM AUTHOR]

A photo essay demonstrating the pitfalls in visual field testing is presented.

We present a case of Aspergillus fumigatus endophthalmitis complicated by necrotizing scleritis in a 68-year-old man with diet-controlled diabetes, after retinal detachment repair. He was initially treated with systemic steroids for surgically induced necrotizing scleritis following routine pars plana vitrectomy. An additional diagnosis of endophthalmitis was made when the patient developed a hypopyon. Repeat vitreous culture isolated Aspergillus fumigatus. Symptoms improved following antifungal treatment leaving the patient with scleromalacia and an advanced postoperative cataract. Fungal scleritis and endophthalmitis are rare complications of intraocular surgery with sight-threatening consequences, and, as this case demonstrates, may even occur concomitantly. The overlapping features of both conditions can make differentiating one from the other difficult. A fungal aetiology should be considered in cases of postoperative scleritis and endophthalmitis that are protracted and refractory to standard therapy. Even in cases of early diagnosis and treatment, visual outcomes in Aspergillus endophthalmitis and scleritis are variable and often disappointing, not infrequently necessitating enucleation of a painful blind eye. [ABSTRACT FROM AUTHOR]

Purpose: To describe characteristics of intraocular inflammation in eyes with active ocular toxoplasmosis and to identify relationships between signs of inflammation, complications (including elevated intraocular pressure [IOP]), other disease features, and host characteristics. Design: Multicenter, retrospective, cross-sectional study. Methods: We reviewed the medical records of 210 patients with toxoplasmic retinochoroiditis at seven international sites (North America, South America, and Europe) for information from the first examination at each site during which patients had active retinal lesions. Signs of inflammation included anterior chamber (AC) cells and flare and vitreous humor cells and haze. Retinal lesion characteristics included size (≤1 disc area [DA] or >1 DA) and presence or absence of macular involvement. Results: AC cells and flare were related to vitreous inflammatory reactions (P ≤ .041). One or more signs of increased inflammation were related to the following factors: older patient age, larger retinal lesions, and extramacular location. In 30% of involved eyes, there was evidence of elevated IOP (despite use of glaucoma medications by some patients); other complications were uncommon. IOP of more than 21 mm Hg was associated with both increased AC cells and elevated flare (both P ≤ .001) and with macular involvement (P = .009). Inflammation seemed to be more severe among patients in Brazil than among those at other sites. Conclusions: There is substantial variation between patients in the severity of intraocular inflammation associated with ocular toxoplasmosis, attributable to multiple host- and disease-related factors. Results suggest that disease characteristics also vary in different areas of the world. Elevated IOP at initial examination reflects the severity of inflammation. [Copyright &y& Elsevier]

Objective: To investigate the clinical features of ocular lesions in Behçet's disease in different countries. Methods: A descriptive questionnaire survey was performed. Results: 25 eye centres in 14 countries returned questionnaires on prevalent cases in 2006. Clinical data were analysed on 1,465 patients with ocular lesions. Recurrent oral aphthous ulcers were reported in 94.5%, skin lesions in 69.5% and genital ulcers in 61.4%. Most of the patients had bilateral and recurrent intraocular inflammation. Poor visual acuity was seen in 18.9% in women, but 24.8% in men (p<0.01). Panuveitis was seen more in men than in women (p<0.01). 23% of the patients had visual acuity equal to or worse than 20/200 at the final visit. The patients with poor vision were more frequently in India, Iran and Japan than in other countries (p<0.01). Conclusions: We report the largest contemporary international case series of patients with ocular involvement in Behçet's disease. Panuveitis was significantly more frequent in men than women, and men tended to have a worse visual prognosis. There were some differences in the clinical pattern of Behçet's disease in different countries. Despite modern treatment, the disease still carries a poor visual prognosis with one-quarter of the patients blind. [ABSTRACT FROM AUTHOR]

Purpose: Reliable information is needed to counsel parents of children with congenital toxoplasmosis regarding the long-term risk of visual impairment resulting from ocular toxoplasmosis.Design: Prospective cohort study of children with congenital toxoplasmosis identified by prenatal or neonatal screening.Methods: After three years of age, ophthalmologists reported the site of retinochoroidal lesions and visual acuity and parents reported visual impairment. An ophthalmologist predicted the child's vision based on the last retinal diagram. Selection biases were minimized by prospective enrollment and data collection, high rates of follow-up, and exclusion of referred cases.Results: Two hundred and eighty-one of 284 infected children who underwent ophthalmic examinations were followed up to a median age of 4.8 years. One in six children (49/281; 17%) had at least one retinochoroidal lesion, two-thirds of whom (32/49; 65%) had a lesion at the posterior pole. In children with retinochoroiditis who had visual acuity measured after 3 years of age, 94% (31/33) had normal vision in the best eye (6/12 Snellen or better), as did 91% of those with a posterior pole lesion (21/23). Analyses based on affected eyes showed that 42% (29/69) had a posterior pole lesion, of which just more than half (15/29, 52%) had normal vision, as did 84% (16/19) of eyes with a peripheral lesion alone. Vision predicted by the ophthalmologist was moderately sensitive (59%) but overestimated impairment associated with posterior pole lesions. Of 44 children with information on acuity, four (9%) had bilateral visual impairment worse than 6/12 Snellen.Conclusions: Severe bilateral impairment occurred in 9% of children with congenital toxoplasmic retinochoroiditis. Half the children with a posterior pole lesion and one in six of those with peripheral lesions alone were visually impaired in the affected eye. [ABSTRACT FROM AUTHOR]

: PurposePediatric uveitis is rare and has been reported to cause increased rates of visual loss compared with adult patients. The reasons for this are unclear. Only one study has been population–based, so the effect of referral bias is not known. We examined the pattern of disease in primary and referral centers to establish the unique characteristics of uveitis in children.: DesignCase control study.: MethodsRetrospective, multicenter, observational study of uveitis starting before the age of 20 years. Two hundred forty-nine patients were recruited from three primary and two referral ophthalmic units. Age-related differences in types of uveitis and systemic disease between hospitals were characterized, as were associations with visual loss.: ResultsThe incidence of uveitis in district hospitals at less than 16 years of age was 4.9/100,000: the most frequent diagnosis was idiopathic uveitis (78%). In referral cohorts the most frequent diagnosis was juvenile idiopathic arthritis-associated uveitis (67%). Other systemic diseases were rare. The most frequent type of uveitis at 0 to 7 years of age was chronic anterior uveitis, posterior uveitis in 8- to 15-year-olds, and acute anterior uveitis in 16- to 19-year-olds. Visual loss (any eye < 6/12) occurred in 17% and was not associated with age, sex, or hospital cohort. It was most frequent in posterior uveitis (25%). Treatment variables were independent predictors of visual loss: systemic treatment 2.2 (1.1– 4.6), surgical intervention 8.2 (3.8–17.6).: ConclusionsIdiopathic uveitis was three times more common in district hospitals. Visual loss was similar to adult uveitis in this study. The increased frequency of severe chronic anterior uveitis in children aged 0 to 7 years and posterior uveitis in older children aged 8 to 15 years accounts for the rate of visual loss seen in previous studies. [Copyright &y& Elsevier]

Behçet disease is a chronic, relapsing-remitting autoinflammatory syndrome with a strong HLA-B*51 association. In this paper, we describe a human cohort of 267 individuals with Behçet disease and 445 matched controls from a tertiary referral center in the U.K. HLA-B*51 was confirmed as a genetic risk factor in this group (p = 0.0006, Bonferroni-Dunn correction for multiple testing [Pc] = 0.0192, odds ratio [OR] 1.92, 95% confidence interval [CI] 1.33-2.76). KIR3DL1/S1 allele-level analysis indicated that low-expressing KIR3DL1/S1 alleles in combination with KIR3DS1 increased the risk of developing Behçet disease (KIR3DL1LOW/KIR3DS1: p = 0.0004, Pc = 0.0040, OR 2.47, 95% CI 1.43-4.25), whereas high-expressing KIR3DL1/S1 alleles in combination with a null-expressing KIR3DL1 reduced the risk of disease (KIR3DL1HIGH/KIR3DL1NULL: p = 0.0035, Pc = 0.0350, OR 0.53, 95% CI 0.33-0.87). Behçet disease can manifest as a purely mucocutaneous disease or can involve other organ systems such as the eyes. In the U.K. cohort studied in this study, KIR3DL1LOW/KIR3DS1 increased the risk of ophthalmic disease (p = 1.2 × 10-5, OR 3.92, 95% CI 2.06-7.47), whereas KIR3DL1HIGH/KIR3DL1NULL reduced the risk of having purely mucocutaneous disease (p = 0.0048, OR 0.45, 95% CI 0.25-0.81). To our knowledge, this is the first analysis of KIR3DL1/S1 allelic variation in Behçet disease and may provide insight into the pathogenic role of HLA-B*51 and its interaction with KIR3DL1/S1. [ABSTRACT FROM AUTHOR]

This is a rare presentation of brain tumour in the region of the lateral geniculate nucleus (LGN) presenting as a homonymous horizontal sectoranopia (HHS). The case highlights that subtle field defects can be asymptomatic and only detected by formal perimetry. Although homonymous sectoranopia is a rare form of visual field defect, it should be recognised as a potential manifestation of potentially significant intracranial pathology. [ABSTRACT FROM AUTHOR]

Introduction: The etiology of Behçet’s disease (BD) is unknown, but widely considered an excessive T-cell mediated inflammatory response in a genetically susceptible host. Recent genome-wide association studies (GWAS) have shown limited number of novel loci-associations. The rarity and unequal distribution of the disease prevalence amongst different ethnic backgrounds have hampered the use of GWAS in cohorts of mixed ethnicity and sufficient sample size. However, novel statistical approaches have now enabled GWAS in admixed cohorts. Methods: We ran a GWAS on 336 BD cases and 5,843 controls. The cases consisted of Western Europeans, Middle Eastern and Turkish individuals. Participants from the Generation R study, a multiethnic birth cohort in Rotterdam, The Netherlands were used as controls. All samples were genotyped and data was combined. Linear regression models were corrected for population stratification using Genomic Principal Components and Linear Mixed Modelling. Meta-analysis was performed on selected results previously published. Results: We identified SNPs associated at genome-wide significant level mapping to the 6p21.33 (HLA) region. In addition to this known signal two potential novel associations on chromosomes 6 and 18 were identified, yet with low minor allele frequencies. Extended meta-analysis reveal a GWS association with the IL12A variant rs17810546 on chromosome 3. Discussion: We demonstrate that new statistical techniques enable GWAS analyses in a limited sized cohort of mixed ethnicity. After implementation, we confirmed the central role of the HLA region in the disease and identified new regions of interest. Moreover, we validated the association of a variant in the IL2A gene by meta-analysis with previous work. These findings enhance our knowledge of genetic associations and BD, and provide further justification for pursuing collective initiatives in genetic studies given the low prevalence of this and other rare diseases. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented discussing a mini case series of spectral domain optical coherence tomography (OCT) findings in bilateral diffuse uveal melanocytic proliferation (BDUMP), a rare paraneoplastic disorder.

An 85-year-old man developed chronic postoperative endophthalmitis after complicated cataract surgery. Visual acuity in the affected eye was hand movements. Slitlamp biomicroscopy showed a hypopyon, superonasal iris nodule, and marked vitritis. An anterior chamber washout, iris biopsy, and intravitreal amphotericin injection were performed. Panfungal polymerase chain reaction of anterior chamber and vitreous samples were positive for Penicillium citrinum . The iris biopsy showed hyphae on Grocott staining. Despite treatment, the patient's acuity deteriorated to light perception and he developed severe intractable pain requiring evisceration. Histological analysis showed diffuse infiltration of hyphae. Penicillium species are fungal organisms that are ubiquitous in the environment and can cause chronic endophthalmitis. They are commonly dismissed as culture contaminants. True infection is confirmed by histological demonstration of fungal invasion. Diagnosis can be aided by iris biopsy if iris nodules are present. Polymerase chain reaction testing was beneficial in identifying the causative organism and should be considered early in endophthalmitis cases. Despite intravitreal and systemic antifungal treatment, the visual prognosis for this condition is variable. Financial Disclosure None of the authors has a financial or proprietary interest in any material or method mentioned. [ABSTRACT FROM AUTHOR]

Background The importance of patient and public involvement (PPI) in healthcare decisions and research is increasingly recognised. This paper describes the aims, delivery, evaluation and impact of a 'Birdshot Day' organised for patients with birdshot uveitis, their carers and healthcare professionals. Methods Delivery of this event involved the close collaboration of patients with a large number of different healthcare professionals. The event's evaluation used established social research methods including qualitative questionnaires pre, post and 6 months following the event. The results were statistically analysed. Results Results indicated that this event significantly educated both patients and professionals. The sense of isolation felt by patients was reduced and networking was developed among all attendees. Patient priorities for research were recorded and invaluable insight into patients' needs for a better quality of life was gained. Conclusions The first undertaking of this novel PPI event achieved all its aims. It became even clearer that fundamental questions remain about birdshot uveitis, including aetiology, pathogenesis, practical clinical issues and impact on quality of life. These questions can only be addressed in partnership with patients. To this end, patients and professionals came together under the banner 'Team Birdshot' and the National Birdshot Research Network was launched. [ABSTRACT FROM AUTHOR]

Objectives. Behçet’s disease (BD) is more severe among young males and disease severity decreases with age. Therefore, the effect of disease activity, gender and age on platelet and neutrophil activation in whole blood taken from patients with BD was investigated.Methods. Using an anti-coagulant Tripotassium ethylenediaminetetra acetic acid (K3EDTA) plus citrate-theophylline-adenosine-dipyridamole (CTAD) (K3EDTA/CTAD) that preserves the degree of platelet activation that exists in vivo, we assessed neutrophil and platelet activation, microparticles, and monocyte and neutrophil–platelet aggregate formation in 43 BD patients using flow cytometry. This is the first description of platelet activation and microparticles in BD patients using this methodology.Results. Inactive [2.78 (0.56)%, P = 0.0009; 3.11 (0.78)%, P < 0.0001] and active [2.28 (0.84)%, P < 0.0001; 3.071 (0.67)%, P = 0.0031] BD patients had significantly higher percentages of CD62P-expressing platelets and CD62P+ platelet microparticles as compared with healthy controls (HCs) [0.84 (0.1)% and 1.23 (0.14)%], respectively. The percentages of CD62P+ platelets and CD62P+ platelet microparticles in female and male BD patients were also significantly higher than those expressed by female and male HCs. The percentages of CD62P+ microparticles were significantly increased in the 20–30-(P = 0.0301) and 31–50-(P < 0.0162) year age ranges, but not in the >50-year age group of BD patients.Conclusion. BD is a rare, chronic multi-systemic vasculitis and interaction of activated platelets with leucocytes has been linked to pathological disorders associated with vascular inflammation. Importantly, this study demonstrates that platelet microparticle activation is increased in BD. Also, this is the first report in which changes in platelet activation in BD are concordant with the observations that BD disease activity diminishes with age. [ABSTRACT FROM PUBLISHER]

Objectives. The initiating cause of Behçet's disease (BD) is unknown, but an aberrant response to infection has been suggested. In this study, single nucleotide polymorphisms in Toll-like receptors (TLRs) and associated molecules that have a sentinel function at mucosal surfaces were analysed in patients with BD.Methods. TLR expression was determined by immunohistochemistry in buccal mucosal tissue from patients with BD, in tissue from patients with lichen planus (LP) or pyogenic granuloma (PG) as disease controls, or from healthy individuals. Using SSP-PCR we analysed SNP in CD14, TLR2, TLR4 and TIRAP (TIR domain-containing adaptor protein) in patients with BD from different geographical regions.Results. TLR expression was increased in buccal lesions from patients with BD compared with healthy controls; however, a similar increase was seen in lesion tissue from patients with LP or PG, suggesting that this was a generalized inflammatory response as opposed to a BD-specific response. SNP analysis showed no association between CD14, TLR2 or TLR4 polymorphisms. However, TIRAP 180Leu was significantly associated with BD in UK, but not Middle Eastern, patients.Conclusion. TLR expression showed no difference in tissue from patients with BD compared with either disease or healthy controls. Likewise, SNPs in TLR genes were no different from healthy controls. The association with the increased function variant of TIRAP suggests that encounter with a pathogen at mucosal sites will lead to increased cytokine production and tissue damage with persistence of mucosal lesions. [ABSTRACT FROM PUBLISHER]

Purpose: To study the effects of intravitreal foscarnet and the clinical differences between varicella zoster virus (VZV) and herpes simplex virus (HSV) induced acute retinal necrosis (ARN). Design: Retrospective comparative case series. Participants: Eighty-one eyes of 74 patients. Methods: A retrospective case note analysis was performed in 2 tertiary referral centers. Main Outcome Measures: Presenting and final visual acuity, and progression to retinal detachment. Results: Thirty-three eyes had HSV-ARN and 48 had VZV-ARN. The average age for HSV-ARN was 34 years and 51 for VZV-ARN (P<0.001). Visual acuity on presentation was similar (P = 0.48), but a larger proportion had better vision (≥20/60) in the HSV-ARN group (52%) than the VZV-ARN group (35%). A greater proportion of eyes with poor vision (≤20/200) was found at the 12-month follow-up in the VZV-ARN group (60%) compared with the HSV-ARN group (35%). A greater degree of visual loss in the VZV-ARN group (0.4 logarithm of the minimum angle of resolution [logMAR]) compared with the HSV-ARN group (0.04 logMAR) was detected (P = 0.016). Retinal detachment was 2.5-fold more common in VZV-ARN (62%) compared with HSV-ARN (24%). When comparing eyes treated with (n = 56) and without (n = 25) intravitreal foscarnet, there was a 40% lower rate in retinal detachment (53.6% vs 75.0%) for VZV-ARN (P = 0.23). The numbers with HSV-ARN were too small for analysis. Conclusions: The results support the difference of outcome in HSV-ARN and VZV-ARN. Therefore, viral identification serves as a key to predicting outcome in these patients. Intravitreal foscarnet seems to be a useful adjunct for the treatment of ARN in that it reduced rate of retinal detachment. Financial Disclosure(s): The authors have no proprietary or commercial interest in any of the materials discussed in this article. [Copyright &y& Elsevier]

The article presents a case study of a 74 year-old man who had a three day history of a red right eye and decreased vision. It is reported that he had no past ophthalmic history. His only past medical history was that he had had a cerebrovascular accident one month. He was diagnosed with ocular ischaemic syndrome (OIS) secondary to occult giant cell arteritis. It occurs when there is reduced flow in both the internal and external carotid arteries.

Abstract: Behçet’s disease (BD) is a multisystem inflammatory disease characterized by recurrent orogenital ulceration, ocular inflammation, and skin lesions. The etiology of the disease is currently unknown but evidence suggests that there is a strong genetic component mediating the chronicity of the disorder. We have examined the association between polymorphisms at position −1082, and −819 in the promoter region of the gene encoding IL-10 in patients with Behçet’s disease from two distinct patient populations. The IL-10 −1082AA genotype was weakly associated with BD when all patients were analyzed as a group (pc = 0.04, OR 1.4, 95% CI 1.1–1.9), but not in the UK or Middle Eastern (ME) cohorts of patients alone compared to local controls. An association with IL-10 −819T was evident in all BD patients, (pc = 0.02, OR 1.5, 95% CI 1.1–2.0), and this was because of an association in the UK but not ME patients (pc = 0.0004, OR 2.1, 95% CI 1.4–3.3). The −1082A/−819T haplotype, which is linked to low production of this cytokine, was not significantly associated with Behçet’s disease. This link between BD, a chronic, relapsing, autoinflammatory condition, and a genotype associated with low IL-10 production provides evidence that abnormalities in the genetic control of cytokine levels may be relevant in influencing the immune response in Behçet’s disease in some patient groups. [Copyright &y& Elsevier]

Retinal vasculitis (RV) is characterised pathologically by migration of leucocytes across the blood–retinal barrier leading to oedema and photoreceptor cell dysfunction. Chemokines are a family of small molecules involved in leucocyte migration. In this study, levels of chemokines were measured in serum from patients with RV and correlated with disease activity and drug treatment. Serum samples (n=100; 25 active, 75 inactive) were obtained from 50 patients with RV, and levels of the chemokines MIP-1α, macrophage inflammatory protein-1β (MIP-1β) and monocyte chemoattractant protein-1 (MCP-1) were measured by ELISA. For longitudinal analysis levels of the same chemokines were measured in six consecutive serum samples from 10 of the above patients. Chemokine levels were correlated with disease activity and current drug treatment for each sample. Sera from 20 healthy individuals were used as control samples. Serum levels of MIP-1β were significantly raised in patients with RV, whether active or not, compared to healthy controls (P=0.04). Levels of MIP-1β and MCP-1 correlated with disease activity in some patients and with prednisolone levels in patients on this treatment alone. MIP-1α levels were not detectable in all samples but were present in significantly more samples from patients with active or inactive RV compared with healthy controls. Serum levels of the chemokines MIP-1β and MIP-1α, but not MCP-1 were raised in patients with retinal vasculitis. Longitudinal analysis suggested that MIP-1β and MCP-1 levels were controlled by drug treatment, particularly prednisolone. These data demonstrate that chemokines are involved in the pathogenesis of RV and may act as novel therapeutic targets. [Copyright &y& Elsevier]

Endogenous bacterial endophthalmitis is a rare but serious condition that occurs when bacteria cross the blood–ocular barrier and multiply within the eye. We provide an overview of endogenous bacterial endophthalmitis by reviewing 267 reported cases and integrating this with our experience of an additional 19 cases. The majority of patients with endogenous bacterial endophthalmitis are initially misdiagnosed and many have an underlying disease known to predispose to infection. This condition is often previously undiagnosed. Blood cultures are the most frequent means of establishing the diagnosis. The most common Gram positive organisms are Staphylococcus aureus, group B streptococci, Streptococcus pneumoniae,and Listeria monocytogenes.The most common Gram negative organisms are Klebsiella spp., Escherichia coli, Pseudomonas aeruginosa, and Neisseria meningitidis. Gram negative organisms are responsible for the majority of cases reported from East Asian hospitals, but Gram positive organisms are more common in North America and Europe. The visual outcome is poor with most cases leading to blindness in the affected eye. Many patients have extraocular foci of infection, with an associated mortality rate of 5%. The outcome of endogenous bacterial endophthalmitis has not improved in 55 years and clinicians need to have a high level of awareness of this commonly misdiagnosed condition. [Copyright &y& Elsevier]

Objective. It has been shown previously that three nucleotide-binding oligomerization domain containing 2 (NOD2) variants (Arg702Trp, Gly908Arg and Lue1007fs) are associated with Crohn's disease (CD), a disorder clinically resembling Behçet's disease (BD). We studied the frequency of these variants in BD patients. Methods. DNA samples of 200 BD patients [59 Caucasians, 139 Middle Easterns (MEs) of Arab descent and 2 Asians] and 520 healthy controls (444 Caucasians and 76 MEs) were genotyped using a Taqman assay. Results. Both the Arg702Trp and Leu1007fs (frameshift) variants were significantly less frequently present among BD patients compared with healthy controls (0.5 vs 5.8%; P<1.10-5 and 0.0 vs 1.8%; P< 0.007, respectively). In the Caucasian subpopulation, Arg702Trp was significantly less frequent in the BD group as compared with the controls (P=0.04); whereas in the ME subpopulation, a trend was observed (P<0.06). Conclusions. Of the three CD-associated single nucleotide polymorphisms, one of the variant NOD2 alíeles, was found to be present significantly less in Caucasian BD patients. [ABSTRACT FROM AUTHOR]

Purpose: Assessing disease activity in Birdshot uveitis can be challenging. The peak time of the 30 Hz flicker photopic electroretinogram (ERG) was shown to be sensitive for detecting retinal dysfunction. ERG testing is not readily available in many eye units. We investigated use of a portable device to obtain recordings in the clinic setting and compared these with conventional recordings. Methods: Patients underwent testing with the portable device (RETeval, LKC Technologies) and conventional equipment (Espion ColorDome, Diagnosys). For portable recordings pupils were undilated and skin electrodes used; for conventional recordings a conductive fibre electrode was used with mydriasis. Flicker ERG peak times were recorded and were deemed abnormal if they exceeded the 95th centile from a healthy cohort (>190 healthy volunteers). Results: 18 patients with Birdshot uveitis underwent recordings. Recordings with the portable device took c. 3 min and were well‐tolerated. Mean (SD) age was 56.3 (12.8) years. For portable recordings, mean (SD) peak times were 30.1 (3.7) and 30.2 (3.9) ms for right and left eyes respectively. For conventional recordings mean (SD) peak times were 31.4 (3.9) and 31.8 (4.0) ms respectively. Peak times were significantly shorter with the portable device (p = 0.026 and 0.002 for right and left eyes). Correlation between devices was high (0.83 and 0.89 for right and left eyes) and highly significant (p < 0.0001). Strength of agreement between the two methods was good (kappa statistic 0.64; 95% CI, 0.36‐0.92). Conclusions: Correlation between the portable and conventional ERG recordings was high with a good level of agreement. Recordings were rapid and well‐tolerated. There were significant differences in peak times between the two methods highlighting the importance of normative data specific to each device. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding a 15-year-old Caucasian boy who was diagnosed with Neuro-Behçet's disease and frosted branch angiitis (FBA).