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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 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]

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]

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

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]

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.

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.