Knowledge and Current Practices in Monogenic Uveitis: An International Survey by IUSG and AIDA Network.

INTRODUCTION: This study aims to explore awareness, knowledge, and diagnostic/therapeutic practices in monogenic uveitis (mU) among uveitis experts. METHODS: This is an explorative, cross-sectional survey study. An anonymous, semi-structured, electronic survey was delivered to uveitis experts from the Autoinflammatory Diseases Alliance (AIDA) Network and International Uveitis Study Group (IUSG). We included respondents answering ≥ 50% of the survey. RESULTS: Seventy-seven participants rated their knowledge of mU as proficient (3.9%), adequate (15.6%), sufficient (16.9%), or poor (63.6%). When asked about the first mU gene they thought of, 60.4% mentioned NOD2, 3.9% mentioned NLRP3 or MEFV, and 49.4% provided incorrect or no answers. Success rates in clinical scenarios varied from 15.6% to 55.8% and were higher for ophthalmologists working in multidisciplinary teams (p < 0.01). Genetic testing was ordered for suspected mU by 41.6% of physicians. The availability of molecular techniques did not significantly differ based on geography (p > 0.05). The public healthcare system ensured a higher percentage of tests prescribed were obtained by patients compared to private insurances (p < 0.00). In terms of disease-modifying anti-rheumatic drugs (DMARDs), tumor necrosis factor-α inhibitors were the most familiar to uveitis experts. The difficulties with off-label therapy procedures were the primary barrier to DMARDs prescription for patients with mU and correlated inversely with the obtained/prescribed drug ratio for interleukin-1 (p < 0.01) and interleukin-6 (p < 0.01) inhibitors. CONCLUSIONS: This survey identifies proficiency areas, gaps, and opportunities for targeted improvements in patients care. The comprehensive outputs may inform evidence-based guidelines, empowering clinicians with standardized approaches, and drive an AIDA Network-IUSG unified effort to advance scientific knowledge and clinical practice.

Clinical Profile of Scleritis Presenting for the First Time in the Elderly.

PURPOSE: To analyze clinical characteristics of scleritis in elderly patients and also compare the data with relatively younger patients with scleritis (<60 years). METHOD: Retrospective analysis of medical records of patients with scleritis who visited a tertiary eye care centre between 2008 and 2018. RESULT: Scleritis in ≥60 years accounted for 3% of the total scleritis cases. The mean age of the patients was 67 ± 6 years, and a female (66%) predominance was noted. Of the 44 elderly patients with scleritis, 48% were 66-70 years of age and 20% were above 70 years of age. Diffuse scleritis was the most common subtypes followed by necrotizing scleritis (35%). Overall, 32% of elderly patients with scleritis had underlying disease and the most common systemic association was granulomatous with polyangiitis. When compared with a subset of patients (<60 years of age), the elderly group showed higher rate of recurrences and complications. CONCLUSION: Scleritis in elderly patients is relatively rare but can have a higher recurrence rate with a increased complications.

Pediatric Sympathetic Ophthalmia: 20 Years of Data From a Tertiary Eye Center in India.

PURPOSE: To examine the clinical profile of sympathetic ophthalmia among the pediatric age group. METHODS: Retrospective review of patients 18 years and younger with sympathetic ophthalmia seen in a tertiary eye care center between 1997 and 2017. RESULTS: Of 20 patients included in the study, 70% were male. The most common inciting event for sympathetic ophthalmia was trauma (85%), followed by vitreoretinal surgery (15%). All patients were treated with systemic steroids. Seventeen patients received additional corticosteroid-sparing immunosuppressive agents, and 4 patients (20%) required more than one immunosuppressive agent. Azathioprine was the most commonly used corticosteroid-sparing immunosuppressive agent. The most common complications were cataract (50%) and ocular hypertension (30%). The mean presenting best corrected visual acuity in the sympathizing eye was 1.15 ± 0.99 logarithm of the minimum angle of resolution (logMAR), which improved to 0.54 ± 1.00 logMAR following treatment. Visual outcome was good (6/12 or better) in 70% of the sympathizing eyes, and 3 of the exciting eyes in the current study had good visual outcomes after the treatment. CONCLUSIONS: Prompt and effective management with corticosteroid-sparing immunosuppressive therapy in children with sympathetic ophthalmia allows favorable control of the disease and retention of good visual acuity. [J Pediatr Ophthalmol Strabismus. 2020;57(3):154-158.].

Intravitreal sustained-release dexamethasone implant for the treatment of persistent cystoid macular edema in ocular syphilis.

With a resurgence of syphilis with human immunodeficiency virus (HIV) infection in last few years, various ocular manifestations of syphilis have been described in literature. This case report described an HIV-positive patient on anti-retroviral therapy who was diagnosed and treated for posterior uveitis secondary to ocular syphilis in the recent past presented to our clinic with cystoid macular edema (CME). CME, which did not respond to periocular corticosteroid, resolved with intravitreal sustained release dexamethasone implant. There was a recurrence CME 9 months later and repeat injection of intravitreal implant showed complete resolution. A long-term follow-up did not reveal reactivation of the infection with intravitreal corticosteroid. Intravitreal sustained release dexamethasone implant can be an effective treatment for refractory CME in patients with regressed syphilitic uveitis.

Choroidal and Retinal Anatomical Responses Following Systemic Corticosteroid Therapy in Vogt-Koyanagi-Harada Disease Using Swept-Source Optical Coherence Tomography.

PURPOSE: To assess structural changes in retina and choroid after systemic corticosteroid therapy in Vogt-Koyanagi-Harada (VKH) disease using swept-source optical coherence tomography (SS-OCT). METHODS: SS-OCT was conducted before treatment and during first-month follow-up in 16 eyes treated with systemic corticosteroids for active VKH. Retina was divided into five zones depending on pretreatment choroidal thickness (CT) of <100, >100 to <200, >200 to <300, >300 to <400 and >400µm, and changes in retinal thickness and CT after treatment in these zones were compared with baseline. RESULTS: Mean CT significantly improved from 83.1±8.75 to 156.4±62.73µm(p = 0.008) in the zone with pre-CT <100µm and significantly decreased from 336.1 ± 17.28 to 266.28 ± 81.39µm(p = 0.008) in the zone with pre-CT > 300µm. CONCLUSIONS: We have shown choroidal remodeling in VKH. SS-OCT can serve as an important noninvasive tool in assessment of treatment response in patients with VKH disease.

Genotypic Detection of Epstein Barr Virus from Clinically Suspected Viral Retinitis Patients in a Tertiary Eye Care Centre, India.

PURPOSE: To evaluate the diagnostic value of PCR on aqueous humour for detection and genotyping of Epstein Bar Virus in patients with viral retinitis. METHODS: 70 AH samples were collected from 20 HIV positive patients with clinically suspected viral retinitis and 25 patients with serpignous choroiditis and 25 AH from patients undergoing cataract surgery. PCR was performed to screen HHV-1 to HHV-5, Mtb and Toxoplasma gondii. Genotype prevalence was confirmed by phylogenetic analysis targetig EBV. RESULTS: EBV was detected in 17 (37.7%) samples. Genotyping to subtype EBV, revealed the circulation of only one subtype (Type 1). PCR results for other infective agents were negative except for the presence of CMV in 5 (11.1%) AH. CONCLUSION: The application of PCR to detect genotypes can be used as an epidemiological tool for clinical management. To our knowledge this is the first report on genotyping of EBV performed on intra ocular samples.

Ocular manifestation of storage diseases.

PURPOSE OF REVIEW: This is an attempt to provide a brief overview of ocular manifestation of storage diseases (lysosomal storage diseases). RECENT FINDINGS: Lysosomal storage disorder is a heterogeneous group of rare disorders characterized by abnormal accumulation of incompletely degraded substances in various tissues and organs. Patients with these kinds of inherited disorder often present with ocular manifestation along with various systemic features. Systemic manifestations including neurological impairment, skeletal deformities, intellectual and cardiac abnormalities, and gastrointestinal problems are quite common. Ocular complication may cause severe reduction in vision and can affect any part of the eye. Corneal opacification of varying severity is frequently seen. Patients can also present with cataract, vitreous degeneration, retinopathy, optic nerve swelling and atrophy, ocular hypertension, and glaucoma. SUMMARY: The majority of these patients have poor vision due to various ocular complications that are often very difficult to monitor and treat.