Pooja Bhat

Pathophysiology of JIA-associated Uveitis

Abstract Juvenile idiopathic arthritis (JIA)-associated uveitis is an intriguing manifestation of JIA and an important contributor of long-term damage. Its pathophysiology is still poorly understood. This review summarizes current concepts. JIA is both a multifactorial and polygenetic disease. In the past 2 decades, multiple studies have indicated that the genetic contribution to both JIA and JIA-associated uveitis is modest. From an ophthalmological point of view, much of the pathophysiological data is derived from studies in experimental uveitis animal models. The pathophysiology of the arthritic manifestations of JIA has been studied extensively in humans. These studies have focused on the principal cells of the adaptive immune system, in particular different subsets of regulatory and effector T cells, as well as on antigen presenting cells or dendritic cells. With advancing technology and advancing knowledge of the underlying mechanisms of JIA-associated uveitis, new targets for therapy might evolve in the coming years.

Changes in Scleral Architecture in Chronic Vogt–Koyanagi–Harada Disease

ABSTRACT Purpose: To describe scleral changes in chronic VKH. Methods: Medical records of patients with chronic VKH were retrospectively reviewed. Change of scleral architecture was defined as progressive posterior bowing on OCT, axial length elongation, and/or increased myopia more than –1.0 D, not explicable by other etiologies. Results: In total, 28 eyes (16 patients) with mean age of disease onset 32.5 ± 14.0 years were included in the study. Disease duration was 15.1 ± 10.2 years. Eight eyes (28.6%) showed progressive scleral architectural changes. Five eyes (18%) developed scleral changes on OCT, not seen on prior imaging (2–12 years earlier). One eye had posterior bowing on OCT with increased axial length, both eyes of a bilateral pseudophake developed increased myopia with increased axial length. Well-circumscribed chorioretinal atrophy within the arcade was associated with progressive scleral change. Conclusions: Progressive scleral change may develop as a late complication of VKH. The association with well-circumscribed chorioretinal atrophy suggests that chronic choroidal inflammation may be responsible.

Response to Kaya and Yildirim’s Letter: “Choroidal Thinning May Change Scleral Architecture”

Wewould like to thank Drs Kaya and Yildirim for their interest in our article. We agree that choroidal atrophy may allow intraocular pressure to affect the sclera. However, choroidal atrophy alone cannot explain the scleral bowing seen in this study, as there was no statistical difference in diffuse choroidal thinning between patients with and without scleral bowing. Moreover, there was no significant difference in intraocular pressure in the group with scleral changes compared with the group without such changes. Chorioretinal atrophywithin the arcade was the only significant association with scleral bowing. Previous reports suggest that the choroid plays a role in regulating scleral remodeling. Given that chorioretinal atrophy may develop after prolonged inflammation, inflammation in the choroid is postulated to affect scleral remodeling, resulting in scleral vulnerability and the potential for posterior scleral bowing. In summary, we agree that intraocular pressure may be one of the factors that cause scleral bowing but, in patients with chronic VKH, the key factor is most likely prolonged choroidal inflammation.

Bilateral Optic Nerve Head Changes in an Asymptomatic Preadolescent Girl

A preadolescent girl presented to the pediatric ophthalmology clinic for evaluation and treatment of possible papilledema, discovered by an outside eye care professional during routine eye examination. Aside from mild hyperopic astigmatism, there was no significant ocular, medical, or family history. The patient denied any change in vision, scotomas, transient visual obscurations, dimming of light, photophobia, or pain. She denied headache, pulsatile tinnitus, weight gain, joint pain, lethargy, decreased appetite, fever, or chills. The patient was not taking any prescription or over-the-counter medicines; however, she had recently used a short-term course of oral antibiotics for a tooth infection. On examination, Snellen visual acuity with correction was 20/20 OD and 20/25 OS. Confrontation visual fields and ocular motility were normal. External examination was unremarkable, and slitlamp examination revealed normal eyelids, conjunctiva, and sclera. There were 1+ anterior chamber white blood cells and trace anterior vitreous cells bilaterally. Posterior segment examination revealed 360° nerve hyperemia with elevation of nasal disc borders bilaterally (Figure 1), without vitreous haze, snowballs, or snowbanks. There was no vascular sheathing, choroidal granulomas, or chorioretinal scarring in the periphery. Right eye A Left eye B

Antibodies in autoimmune retinopathy

ABSTRACT Introduction: Autoimmune retinopathy (AIR) is a rare condition characterized by profound vision loss, presence of anti-retinal antibodies and association with systemic malignancy within the paraneoplastic retinopathy subtype. This review focuses on the different types of autoimmune retinopathies, their clinical features and diagnosis with emphasis on the different types of anti-retinal antibodies and their significance. Areas covered: Cancer associated and melanoma associated retinopathy, as well as non-paraneoplastic autoimmune retinopathy, along with different anti-retinal antibodies in each subtype. A PubMed literature search was carried out using keywords: autoimmune retinopathy, cancer-associated retinopathy, melanoma-associated retinopathy, paraneoplastic retinopathy, and anti-retinal antibodies. Expert opinion: Currently, much remains to be discovered about autoimmune retinopathy. The condition may present with varying clinical features, but commonly recognized manifestations such as photopsias, visual field defects and ERG changes have been identified. The presence of circulating anti-retinal antibodies has been considered essential to the diagnosis of AIR however no universally standardized assays exist. Additionally, the clinical validity and pathogenic role of some of the anti-retinal antibodies is yet to be determined. Immunomodulatory agents, either singly or in combination, have been tried but no therapy has shown a consistent, long-term effect.