Paediatric autoimmune and autoinflammatory conditions associated with uveitis

Childhood uveitis comprises a collection of heterogenous ocular phenotypes which are associated with a diverse range of childhood autoimmune and autoinflammatory disorders. Of these genetic and/or acquired disorders, juvenile idiopathic arthritis is the most common, affecting 30-80% of children with uveitis. Up to a third of children with uveitis have ‘isolated’ idiopathic disease and do not have an associated systemic disease which manifests in childhood. However, uveitis may be the presenting manifestation of disease; thus, the apparently well child who presents with uveitis may have isolated idiopathic disease, but they may have an evolving systemic disorder. The diagnosis of most of the associated disorders is reliant on clinical features rather than serological or genetic investigations, necessitating detailed medical history taking and systemic examination. Adequate control of inflammation is key to good visual outcomes, and multidisciplinary care is key to good broader health outcomes

Long Term Outcomes of Pediatric Idiopathic Intermediate Uveitis

Purpose: To describe the course of childhood-onset intermediate uveitis without associated systemic disease, and investigate determinants of outcomes. / Design: A retrospective clinical cohort study. / Setting: Institutional. / Patients: 125 children (221 eyes) (aged 16 years and under). / Main Outcomes and Measures: Outcomes of interest were visual acuity, severity of inflammation, and the occurrence of sight-threatening complications. Variables examined included age and clinical findings at presentation, treatment, and duration of follow-up. Multivariable analysis was undertaken to investigate potential predictors of outcomes. / Results: Median follow-up duration was 57 months. At presentation, best-corrected visual acuity worse than 20/160 was recorded in 11 (4.4%) eyes, and significant vitreous haze (≥2+SUN) in 35 (14%) eyes. Corticosteroid-sparing agents were used in 41 children (33%), with methotrexate most commonly used (27 children, 21.6%). The most frequent complications were raised intraocular pressure n=65 (29.4%), cataract n=41 (18.5%), and cystoid macular edema n=29 (13.1%). At the last visit, 116 (92.8%) patients achieved best-corrected vision of 20/40 or better with quiescent uveitis. The absence of the use of a steroid-sparing immunomodulatory agent was the strongest predictive factor for the development of new macular edema (OR 6.3, 95% CI 2.3 – 16.9, p<0.001) or glaucoma (OR, 6.6, 95% CI 2.5 – 17.9, p<0.001) over the period of observation. / Conclusions: The visual outcomes of childhood-onset idiopathic intermediate uveitis are favorable. The frequency of sight-threatening sequelae of inflammation, which confer a life-long risk of further visual loss, is high. The use of immunomodulatory therapy is associated with a lower risk of developing macular edema and ocular hypertension

Intermediate uveitis and multiple sclerosis: to scan or not to scan.

The investigation and management of inflammatory eye disease is complex and relies on detailed history taking and exam-ination. It is not therefore unexpected that many patients are over-investigated. A question that has caused much debate in the past is whether patients with inter-mediate uveitis (IU), particularly young Caucasian women with no neurological symptoms, should have routine neuroima-ging to ‘screen ’ for multiple sclerosis (MS). In this editorial, we discuss the key issues related to the clinical presentation of IU, its association with MS and the rationale for investigation; in particular

The Use of Topical Non-steroidal Anti-inflammatory Drugs for Uveitic Cystoid Macular Edema.

The use of topical non-steroidal anti-inflammatory drugs in the management of uveitic cystoid macular edema is controversial. In this letter, we review the literature and report our results from a retrospective case series of 281 patients taking topical nepafenac 0.1% for uveitic cystoid macular edema between 2010 and 2016. Over a 4-month period, there was a modest improvement in best-corrected visual acuity (0.66 ± 3.7 logMAR) and central macular thickness (69.8 ± 132.5 μm). Patients with idiopathic anterior uveitis and HLA-B*27-associated anterior uveitis recovered more vision while taking topical nepafenac 0.1%, than those with other uveitides, however, this was not statistically significant. There is a need for controlled prospective studies to evaluate the efficacy of topical non-steroidal anti-inflammatory drugs in the management of uveitic cystoid macular edema. At present, clinicians have little evidence on which to base practice

Intralesional steroid for orbital manifestations of Rosai-Dorfman disease.

This work was kindly funded by the Frost TFC Charitable Trust and the Royal College of Ophthalmologists Fellowship

Gamma Delta (gamma delta) T Cells and Their Involvement in Behcet's Disease

Behçet’s disease (BD) is a multisystem inflammatory disorder characterized by orogenital ulcerations, ocular manifestations, arthritis, and vasculitis. The disease follows a relapsing-remitting course and its pathogenesis is unknown. Genetic predisposition and immune-dysregulation involving gamma delta (γδ) T cells are reported to have a role. γδ T cells are atypical T cells, which represent a small proportion of total lymphocytes. They have features of both innate and adaptive immunity and express characteristics of conventional T cells, natural killer cells, and myeloid antigen presenting cells. These unconventional T cells are found in the inflammatory BD lesions and have been suggested to be responsible for inducing and/or maintaining the proinflammatory environment characteristic of the disease. Over the last 20 years there has been much interest in the role of γδ T cells in BD. We review the literature and discuss the roles that γδ T cells may play in BD pathogenesis

Endogenous endophthalmitis and liver abscesses.

We present a case of endogenous endophthalmitis secondary to liver abscesses, in a patient with no previous medical comorbidities or risk factors for immunosuppression. The patient presented with acute painless loss of vision and feeling generally unwell. Investigations revealed Streptococcus anginosus-constellatus bacteraemia, and evidence of diverticular disease that likely predisposed to the liver abscesses. Due to prompt diagnosis and administration of antibiotics, the patient had a good visual outcome. This case highlights the importance of being aware of endogenous endophthalmitis, as early diagnosis and prompt administration of antibiotics will optimise visual outcomes