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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]

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]

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]

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]

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]

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]

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]