Beyond dry eye: The greater extent of Sjögren's systemic disease symptoms, the impact of COVID-19 and perceptions towards telemedicine identified through a patient co-designed study.

BACKGROUND: Sjögren's ('SHOW-grins') is a chronic debilitating autoimmune disease characterised by dry eyes and dry mouth, secondary to reduced exocrine function of both the lacrimal and salivary glands. The persistent, severe and serious systemic complications of Sjögren's are poorly understood and often unappreciated, resulting in significant morbidity and treatment burden. This study aimed to explore the experiences of those living with Sjögren's, specifically access to healthcare and attitude towards telemedicine. Additionally, we sought to collect information regarding the impact of the pandemic on their quality of life (QoL). METHODS: One hundred and ninety-four individuals attended an Irish Sjögren's Webinar. Attendees were invited to participate in two online surveys after the webinar. The first survey gathered information related to demographics, disease and experiences during the COVID-19 pandemic. A combination of bespoke items and validated questionnaires (EULAR Sjögren's Syndrome Patient Reported Index [ESSPRI], COVID-19 Impact on Quality of Life [COV19-QoL]) was used. The second survey consisted of a shortened Telehealth Usability Questionnaire. Both were prepared in collaboration with a patient advocate. RESULTS: Survey 1: n = 76; response rate = 39.2%. Thirty-one respondents (41.4%) to survey 1 reported a delay of ≥5 years between the onset of symptoms and diagnosis. Dry mouth was the most common symptom experienced (76.8%, n = 63), followed by dry eye (74.4%, n = 61), fatigue (57.3%, n = 47) and joint pain (53.7%, n = 44), but a range of other symptoms were also reported. COV19-QoL results indicated that the pandemic had a detrimental effect on participants' overall QoL (4.0 ± 1.0) and physical health (4.0 ± 0.8) in particular. COV19-QoL and ESSPRI scores were moderately correlated (0.36, p = .002). Over 70% of respondents had a medical appointment cancelled, delayed or rescheduled (n = 60). Survey 2: n = 57; response rate = 29.4%. Those that had interacted with telemedicine reported largely positive experiences with the virtual model. CONCLUSION: Clinicians should be aware of the range of symptoms experienced by patients with Sjögren's beyond those of sicca (dry eye and dry mouth) and fatigue. COVID-19 has negatively influenced the self-reported health and well-being of those with Sjögren's, particularly those with higher symptom scores. It is vital that optimised telemedicine models are implemented to ensure continuity in the provision of healthcare for those with chronic illness such as Sjögren's and in preparation for possible future pandemics. PATIENT OR PUBLIC CONTRIBUTION: A group of people living with Sjögren's co-designed the structure and content of the webinar where the survey was shared. A public and patient involvement (PPI) contributor also collaborated in the selection of questionnaires used in the study, ensuring that the questions asked would best reflect the priorities of patients. They contributed to the writing of this manuscript as co-authors. Additionally, the research team and Sjögren's patients who contributed to this work have gone on to establish Sjögren's Research Ireland, a collaboration between patient advocates, researchers and PPI facilitators.

Peripheral blood immune cell profiling of acute corneal transplant rejection.

Acute rejection (AR) of corneal transplants (CT) has a profound effect on subsequent graft survival but detailed immunological studies in human CT recipients are lacking. In this multi-site, cross-sectional study, clinical details and blood samples were collected from adults with clinically diagnosed AR of full-thickness (FT)-CT (n = 35) and posterior lamellar (PL)-CT (n = 21) along with Stable CT recipients (n = 177) and adults with non-transplanted corneal disease (n = 40). For those with AR, additional samples were collected 3 months later. Immune cell analysis was performed by whole-genome microarrays (whole blood) and high-dimensional multi-color flow cytometry (peripheral blood mononuclear cells). For both, no activation signature was identified within the B cell and T cell repertoire at the time of AR diagnosis. Nonetheless, in FT- but not PL-CT recipients, AR was associated with differences in B cell maturity and regulatory CD4+ T cell frequency compared to stable allografts. These data suggest that circulating B cell and T cell subpopulations may provide insights into the regulation of anti-donor immune response in human CT recipients with differing AR risk. Our results suggest that, in contrast to solid organ transplants, genetic or cellular assays of peripheral blood are unlikely to be clinically exploitable for prediction or diagnosis of AR.

Telemedicine Service for Keratoconus Monitoring: Patient Satisfaction and Prospects for Further Expansion.

Introduction: During the COVID-19 pandemic, keratoconus patient care moved from in-person clinics to virtual care. We surveyed patient satisfaction with the new virtual clinic model. Methods: We assessed the views of keratoconus patients enrolled in the novel virtual service between June 1 and July 31, 2020, in individual structured telephone interviews using Likert questions. Results: Of the 88 patients enrolled, the opinions of 69 patients could be evaluated (78.4%). Compared with previous in-person visits, mean waiting times for diagnostic examinations dropped from 43 (±32) min to 4 (±3) min (p < 0.001). The majority of patients (68; 99%) were satisfied or very satisfied with the overall service irrespective of the communication channel (telephone or video). A majority also indicated a desire to continue attending the virtual keratoconus clinic after the pandemic and supported the idea of decentralized sites for future diagnostic measurements. Discussion: A novel virtual service to monitor keratoconus progression was well received and was associated with shorter waiting times. There was a strong interest on the part of patients to further develop the virtual keratoconus clinic. Conclusions: This study demonstrates that keratoconus patients managed very well the conversion from in-person to virtual care. A solid majority of keratoconus patients also supported further expansion of the virtual consultations to a completely decentralized telemedicine model.

Performance characteristics and predictors of temporal artery ultrasound for the diagnosis of giant cell arteritis in routine clinical practice in a prospective cohort.

OBJECTIVES: The diagnosis of giant cell arteritis (GCA) is primarily a clinical one. Temporal artery (TA) ultrasound (US) has been proposed as a new diagnostic tool. We aimed to assess the performance characteristics of TA US in routine clinical practice. METHODS: All patients presenting with suspected GCA to our institution are recruited to a prospective registry. Patients who had both a TA US and biopsy (TAB) performed at the time of presentation were included in the current study. The performance characteristics of TA US was compared to physician diagnosis at six months following presentation. Predictive factors for a positive TA US were explored in univariate and multivariable logistic regression analyses. RESULTS: 162 patients were included, 123 (76%) with GCA. Mean (SD) duration of glucocorticoid therapy was 6.6 days (19.4) at the time of TA US. TA US had a sensitivity of 52.8% (95%CI 43.7, 61.9) and specificity of 71.8% (95%CI 54.9, 84.5) for the diagnosis of GCA. Glucocorticoid duration did not significantly impact the results. A sequential strategy of TA US followed by TAB in the case of a negative US had a sensitivity of 78.9% (95%CI 70.1, 85.5) and specificity of 71.8% (95%CI 54.9, 84.5), equivalent to a simultaneous testing strategy. The only factor independently predictive of a positive TA US was male sex (OR 5.53, 95% CI 2.72 to 11.22, p<0.001). CONCLUSIONS: TA US is potentially useful in the diagnosis of GCA; however, interpretation of its results requires knowledge of the performance characteristics in the target population.

Interleukin 12 and interleukin 23 play key pathogenic roles in inflammatory and proliferative pathways in giant cell arteritis.

OBJECTIVES: The pathogenesis of giant cell arteritis (GCA) remains unclear. TH1 and TH17 pathways are implicated, but the proximal initiators and effector cytokines are unknown. Our aim was to assess the role of interleukin 12 (IL-12) and interleukin 23 (IL-23) in GCA pathogenesis. METHODS: IL-12 and IL-23 expression were quantified by immunohistochemistry in temporal artery biopsies (TABs). Temporal artery (TA) explant, peripheral blood mononuclear cell (PBMC) and myofibroblast outgrowth culture models were established. PBMCs and TA explants were cultured for 24 hours in the presence or absence of IL-12 (50 ng/mL) or IL-23 (10 ng/mL). Gene expression in TA was quantified by real-time PCR and cytokine secretion by ELISA. Myofibroblast outgrowths were quantified following 28-day culture. RESULTS: Immunohistochemistry demonstrated increased expression of interleukin 12p35 (IL-12p35) and interleukin 23p19 (IL-23p19) in biopsy-positive TAs, localised to inflammatory cells. IL-12p35 TA expression was significantly increased in those with cranial ischaemic complications (p=0.026) and large vessel vasculitis (p=0.006). IL-23p19 TA expression was increased in those with two or more relapses (p=0.007). In PBMC cultures, exogenous IL-12 significantly increased interleukin 6 (IL-6) (p=0.009), interleukin 22 (IL-22) (p=0.003) and interferon γ (IFN-γ) (p=0.0001) and decreased interleukin 8 (IL-8) (p=0.0006) secretion, while exogenous IL-23 significantly increased IL-6 (p=0.029), IL-22 (p=0.001), interleukin 17A (IL-17A) (p=0.0003) and interleukin 17F (IL-17F) (p=0.012) secretion. In ex vivo TA explants, IL-23 significantly increased gene expression of IL-8 (p=0.0001) and CCL-20 (p=0.027) and protein expression of IL-6 (p=0.002) and IL-8 (p=0.004). IL-12 (p=0.0005) and IL-23 (p<0.0001) stimulation increased the quantity of myofibroblast outgrowths from TABs. CONCLUSION: IL-12 and IL-23 play central and distinct roles in stimulating inflammatory and proliferative pathways relevant to GCA pathogenesis.

A novel evidence-based detection of undiagnosed spondyloarthritis in patients presenting with acute anterior uveitis: the DUET (Dublin Uveitis Evaluation Tool).

BACKGROUND: To date, there are no formal guidelines or referral pathways for acute anterior uveitis (AAU) patients developed or endorsed by any international or national societies. The objective of our study was to develop and validate an assessment algorithm for referral from ophthalmologists of appropriate AAU patients to rheumatology that will aid the early diagnosis of the spondyloarthropathy (SpA). METHODS: All consecutive patients attending the emergency department of local ophthalmology hospital with AAU, but who did not have a known diagnosis of SpA, were eligible to participate in this study. Patients with any other known cause of AAU were excluded. Two independent cohorts were enrolled. Test algorithm and Dublin Uveitis Evaluation Tool (DUET) algorithm (revised form of test algorithm) were used in these cohorts to identify patients as SpA suspects and non-SpA controls, respectively. RESULTS: STUDY PHASE-1. ALGORITHM DEVELOPMENT COHORT (n=101): After rheumatologic evaluation of the entire cohort, 41.6% (n=42) had undiagnosed SpA. Our test algorithm was noted to have: sensitivity 100% and specificity 53.5%. Further regression analysis resulted in the development of the DUET algorithm which made the following improvements: sensitivity 95%, specificity 98%, positive likelihood ratio (LR) 56.19, and negative LR 0.04. STUDY PHASE-2. DUET ALGORITHM VALIDATION COHORT (n=72): After rheumatologic evaluation of the cohort, 40% (n=29) were diagnosed with SpA, with the following performance of DUET algorithm-sensitivity 96%, specificity 97%, positive LR 41.5 and negative LR 0.03. CONCLUSIONS: Approximately 40% of patients presenting with idiopathic AAU have undiagnosed SpA. A simple to apply algorithm is described with excellent sensitivity and specificity.

Isolation of microRNA from conjunctival impression cytology.

Impression cytology (IC) is an easy and safe technique that has been used in the past for harvesting epithelial cells from the cornea and conjunctiva for various applications including histology, immunohistology and molecular studies. Previous investigations have shown the usage of different types of membranes for the purpose of investigating pathophysiology and staging of diseases. This contributes to a better understanding of ocular surface conditions and helps to provide information for diagnosis, therapeutic options and prognosis. Recently, there has been a shift of focus in research towards understanding the contribution of microRNAs (miRs) to ocular disease. Thus far, impression cytology has been explored for measuring gene expression but not for quantifying miR expression. This study describes how miRs and mRNA can be isolated from conjunctival epithelial cells obtained by impression cytology and determines the optimum membrane and technique for this purpose. The IC technique was optimized using Biopore, Immobilon-P(SQ) and Millicell Hanging Cell Culture Insert membranes on healthy controls. miRs and mRNAs were isolated from the conjunctival epithelial cells (CEC) obtained and measured. Biopore membrane provided the optimum yield of miRs (38.8 ng/µL ± 10.8) and mRNA (155.3 ng/µL ± 20.1) as well as subjectively found to be best tolerated with minimum discomfort. Appreciable levels of miRs and mRNAs were detected from the CEC from healthy controls, confirming that it is possible to isolate miR and mRNA from CEC. Here, we give a detailed description of the application of conjunctival impression cytology to isolate miRs and the convenience of the technique by using the best membrane available. This method can be readily adopted in both clinical and laboratory settings. This technique will facilitate the measurement of miRs to improve our understanding of the pathogenesis of ocular surface conditions as well as potentially identifying novel therapeutic targets.

Proteomics in uveal melanoma.

Uveal melanoma is the most common primary intraocular malignancy in adults, with an incidence of 5-7 per million per year. It is associated with the development of metastasis in about 50% of cases, and 40% of patients with uveal melanoma die of metastatic disease despite successful treatment of the primary tumour. The survival rates at 5, 10 and 15 years are 65%, 50% and 45% respectively. Unlike progress made in many other areas of cancer, uveal melanoma is still poorly understood and survival rates have remained similar over the past 25 years. Recently, advances made in molecular genetics have improved our understanding of this disease and stratification of patients into low risk and high risk for developing metastasis. However, only a limited number of studies have been performed using proteomic methods. This review will give an overview of various proteomic technologies currently employed in life sciences research, and discuss proteomic studies of uveal melanoma.

Education in focus: Significant improvements in student learning and satisfaction with ophthalmology teaching delivered using a blended learning approach.

PURPOSE: This study aimed to measure student satisfaction with a revised ophthalmology delivery format, which due to the pandemic had previously relied on a remote online flipped classroom (OFC) format compared to a blended learning format. This educational strategy combined online learning with in-person seminars and practical patient centred sessions. Our previous investigations demonstrated a significant lack of student satisfaction with a curriculum solely reliant on a remote OFC, as such we hypothesised that a blended learning approach would result in improved levels of student satisfaction and knowledge gain. METHODS: Non-randomised intervention study of two groups; group 1 = OFC group and group 2 = BL group, compared perspectives of 4th year ophthalmology students using a validated course evaluation questionnaire (CEQ). RESULTS: A total of 59 students from the BL group (n = 257; response rate = 23.0%) and 28 from the OFC group agreed to participate in the study (n = 114; response rate = 24.6%). Participants in the BL group felt it was easier to determine the standard of work that was expected (77.42% v 60.71%) and demonstrated significantly increased satisfaction with staff motivation of students (95.16% v 64.29%, p <0.001) and provision of feedback (74.19% v 46.43%, p = 0.004), compared to the OFC group. Furthermore, students in the BL group also felt the course significantly improved their analytical skills (64.52% v 42.85%, p = 0.023) and ability to work as part of team (69.36% v 25%, p <0.001) as well as reporting reduced dissatisfaction with the level of choice afforded in terms of how they would learn (33.88% v 60.71%, p = 0.31) and the how they were assessed (59.68% v 89.28%, p = 0.004). No evidence of a statistical difference in exam score was observed. CONCLUSIONS: The COVID-19 pandemic necessitated an unavoidable pivot to online and distance learning, to meet the challenges presented by government mandates and social distancing requirements. Since many of these directives have been reversed, it is important to evaluate the effectiveness and learner perceptions' of the online and distance learning interventions. In this study we demonstrated a significant student preference for BL compared to the OFC approach, with comparable student performances determined by MCQ examinations. Our findings suggest a preference for reintroducing in-person and patient engagement activities in post-pandemic health professions education.

Outcomes after reversed corneal graft rejection: a report from the European VISICORT project.

OBJECTIVE: This study aims to describe the outcome of corneal grafts, both low risk and high risk, after successfully reversed immunological rejection. METHODS: Datasets on reversed rejection episodes in penetrating and endothelial keratoplasties between 2014 and 2019 (n=876) were extracted from the Adverse Immune Signatures and their Prevention in Corneal Transplantation database, which contains the prospectively and consecutively collected corneal transplants from five European centres. Stratified by the preoperatively determined risk status for immunological rejection, the outcome parameters analysed included visual acuity, intraocular pressure, endothelial cell density and central corneal thickness before and after reversed rejection episodes. RESULTS: Fourty-seven (52%) out of a total of 91 identified rejection episodes were successfully reversed and were available for analysis (23 penetrating and 24 endothelial keratoplasties). No statistically significant change was found for any of the parameters studied between the values before and the values 3 months after the rejection episode, irrespective of the preoperative risk status. CONCLUSION: The outcome of corneal grafts that survive immunological rejection may be clinically indistinguishable from the state before immunological rejection, irrespective of graft type and risk status. These findings support clinicians by providing information on prognosis after reversed rejection episodes and by giving patients realistic expectations regarding the outcome.

Levamisole-Adulterated Cocaine-Induced Mucous Membrane Pemphigoid: Case Reports and Literature Review.

PURPOSE: The aim of this study was to report 2 cases of levamisole-adulterated cocaine-induced mucous membrane pemphigoid. METHODS: This study is a review of case reports and literature. RESULTS: Two patients presented with bilateral severe purulent conjunctivitis, corneal ulceration, and rapidly progressive forniceal shortening. Both patients were active cocaine users. A complete blood analysis showed a positive antineutrophil cytoplasmic antibody immunofluorescence with a mixed perinuclear antineutrophil cytoplasmic antibody and cytoplasmic-staining antineutrophil cytoplasmic antibody pattern. Direct immunofluorescence examination of conjunctival tissue showed linear deposition of component 3 and immunoglobulins at the basal membrane. A diagnosis of levamisole-adulterated cocaine-induced mucous membrane pemphigoid was made. In case 1, this suspicion was confirmed by investigating remnants of cocaine on the patient's debit card using mass spectrometry, which contained traces of levamisole. In both cases, aggressive immunosuppressive therapy combining systemic corticosteroids and rituximab was able to control the disease. However, by the time these therapies were initiated, significant corneal injury had occurred requiring corneal grafts in both patients. CONCLUSIONS: Given the rising abuse of cocaine, it is important that ophthalmologists are made aware of its association with severe atypical cicatricial conjunctivitis. To the best of our knowledge, we present the first case proving the causal relationship between levamisole and ocular cicatricial pemphigoid.

Rheumatoid arthritis-associated corneal ulceration: mortality and graft survival.

PURPOSE: To investigate mortality and graft survival in patients undergoing penetrating keratoplasty (PKP) for rheumatoid arthritis-associated corneal ulceration (RACU), Fuchs' endothelial dystrophy (FED), and pseudophakic bullous keratopathy (PBK). DESIGN: Case-control study. PARTICIPANTS AND CONTROLS: Patients listed on the UK Transplant Registry who had undergone a PKP for RACU, FED, or PBK between January 4, 1999, and January 4, 2006. Comparative standardized mortality ratios (SMRs) and causes of death were obtained from the Office for National Statistics. METHODS: Outcome data were collected from the UK Ocular Tissue National Transplant database and supplementary questionnaires at transplantation and at 1, 2, and 5 years. Institutional review board approval for the National Health Service Blood and Transplant to undertake the study was obtained. MAIN OUTCOME MEASURES: Mortality and graft survival. RESULTS: A total of 3665 patients were included: RACU (117), PBK (1701), and FED (1847). Five-year survival of patients with RACU was 42% (95% confidence interval [CI], 26-56) compared with 76% (95% CI, 72-78) for FED and 55% for PBK (95% CI, 50-60; P < 0.01). The SMRs for female and male patients with RACU were 43.5 (95% CI, 19.5-63.3) and 12.2 (95% CI, 7.1-19.5), respectively, in comparison with 1.84 and 1.45 for patients with RA, respectively (P < 0.01). There were no significant differences in the causes of death among patients with RACU, FED, or PBK (P > 0.9), with infection the most common cause. The 5-year graft survival rate was 48% (95% CI, 32-62) for RACU, 59% (95% CI, 56-62) for PBK, and 84% (95% CI, 82-86) for FED (P < 0.01). CONCLUSIONS: Mortality and ocular morbidity were significantly increased in patients with RACU. Accelerated immunosenescence should be considered in the differential diagnosis of patients presenting with RACU, and a multidisciplinary approach to management is required.

Moraxella nonliquefaciens-associated infectious scleritis.

Infectious scleritis is a rare disease entity with potentially devastating visual sequelae. Here we present the clinical history, work-up and aetiology of an unusual case of infectious scleritis.

Isolated Ocular Stevens-Johnson Syndrome Caused by Lymecycline in a Patient with Underlying Ulcerative Colitis.

Stevens-Johnson syndrome (SJS) and the more severe variant, toxic epidermal necrolysis (TEN), are a spectrum of mucocutaneous reactions with potentially devastating ocular consequences. Ocular complications occur in about 70% of patients with Stevens-Johnson syndrome, and 35% continue with chronic disease. We report an unusual presentation of isolated ocular Stevens-Johnson syndrome in a patient with recently diagnosed ulcerative colitis being treated with Infliximab. The case had an insidious and atypical onset and represented a diagnostic dilemma. The diagnosis was more difficult, due to the fact that the inciting agent had long been stopped. Severe bacterial conjunctivitis such as that caused by Chlamydia Trachomatis, Corynebacterium diphtheria, and Neisseria Gonorrhea can cause forniceal shortening and symblepharon; this diagnosis was ruled out with microbiological swabs. A conjunctival biopsy was the key to diagnosis. Treatment involved high-dose IV steroids and dual immunosuppression with Infliximab and mycophenolate mofetil. We sought to employ interventions with the greatest impacts on our patient's condition. Our experience contributes to the growing evidence supporting intensive ophthalmic management of SJS to prevent long-term vision loss.

How has medical student learning changed with the pivot to online delivery of ophthalmology in the pandemic?

PURPOSE: This study aimed to measure stakeholder satisfaction with our usual delivery format, which previously relied on a blend of didactic lectures and clinical skills sessions compared to a revised format, which had more emphasis on online learning. We hypothesised that the online flipped classroom (OFC) would facilitate delivery of content in the wake of the pandemic, and result in improved levels of student satisfaction and knowledge gain. DESIGN: Non randomised intervention study. Group 1 = Traditional delivery (TD) and Group 2 = OFC group. METHODS: A validated course evaluation questionnaire (CEQ) compared perspectives of teaching faculty (n = 5) and students with the traditional delivery (TD) of the 4th year ophthalmology clinical attachment and an OFC approach (TD n = 129 v OFC n = 114). RESULTS: The OFC group (n = 114; response rate = 24.6%) reported significantly reduced satisfaction with staff motivation of students and provision of feedback, compared to TD (n = 129; response rate = 17.8%). OFC students also felt it was harder to determine what standard of work was expected and found the course less beneficial at helping develop problem-solving skills. Students were dissatisfied with the level of choice afforded by the OFC, specifically how they would learn and assessment options. No significant difference in exam score was observed between the TD and OFC groups. For faculty (n = 5), there was no evidence of a difference between OFC and TD. CONCLUSIONS: Students indicated a preference for the TD compared to the OFC approach. However, both delivery approaches led to comparable student performances as determined by MCQ examination.

Impact of adalimumab in patients with active non-infectious intermediate, posterior, and panuveitis in real-life clinical practice: HOPE study.

BACKGROUND/AIM: This study evaluated real-life adalimumab impact in patients with active non-infectious intermediate, posterior, or panuveitis (NIIPPU). METHODS: Adults with active NIIPPU received adalimumab in this prospective, observational study (06/2017-04/2020). Patients were evaluated at baseline (V0) and four follow-up visits over 12 months (V1-V4). PRIMARY ENDPOINT: proportion of patients achieving quiescence (anterior chamber (AC) cells grade and vitreous haze (VH) grade≤0.5+ in both eyes, no new active chorioretinal lesions) at any follow-up visit. Secondary endpoints: proportion of patients achieving quiescence at each visit; proportion of patients maintaining response; and proportion of patients with flares. Workability, visual function, healthcare resource utilisation, and safety were evaluated. RESULTS: Full analysis set included 149 patients. Quiescence at any follow-up visit was achieved by 129/141 (91%) patients. Quiescence at individual visits was achieved by 99/145 (68%), 110/142 (77%), 102/131 (78%), and 99/128 (77%) patients at V1-V4, respectively. Number of patients in corticosteroid-free quiescence increased from 51/147 (35%; V1) to 67/128 (52%; V4; p<0.05). Proportion of patients with maintained response increased from 89/141 (63%; V2) to 92/121 (76%; V4; p<0.05) and proportion of patients with flare decreased from 25/145 (17%; V1) to 13/128 (10%; V4; p=0.092). Workability and visual function improved throughout the study. Proportion of patients with medical visits for uveitis decreased from 132/149 (89%; V0) to 27/127 (21%; V4). No new safety signals were observed. CONCLUSION: These results demonstrated adalimumab effectiveness in improving quality of life while reducing economic burden of active NIIPPU.

Differences in minimal disease knowledge of keratoconus patients: results from an international survey.

BACKGROUND/AIMS: The objective of this multicentre, multinational, prospective study was to assess the level of basic understanding that individuals with keratoconus possessed about their condition. METHODS: We recruited 200 active keratoconus patients who were under regular review, and cornea specialists established a standard of 'minimal keratoconus knowledge' (MKK) that included an understanding of the definition, risk factors, symptoms and treatment options for the condition. We collected data from each participant regarding their clinical characteristics, highest level of education, (para)medical background and experiences with keratoconus within their social circle, and calculated the percentage of MKK attained by each patient. RESULTS: Our findings revealed that none of the participants met the MKK standard, with the average MKK score being 34.6% and ranging from 0.0% to 94.4%. Furthermore, our study showed that patients with a university degree, previous surgical intervention for keratoconus or affected parents had a higher MKK. However, age, gender, disease severity, paramedical knowledge, disease duration and best-corrected visual acuity did not significantly affect the MKK score. CONCLUSIONS: Our study demonstrates a concerning lack of basic disease knowledge among keratoconus patients in three different countries. The level of knowledge exhibited by our sample was only one-third of what cornea specialists would typically anticipate from patients. This highlights the need for greater education and awareness campaigns surrounding keratoconus. Further research is needed to determine the most efficient approaches for enhancing MKK and subsequently improving the management and treatment of keratoconus.

Management of Keratoconus in Down Syndrome and Other Intellectual Disability.

PURPOSE: The purpose of this study was to assess an intellectual disability (ID) cohort with keratoconus (KC) regarding ophthalmic (visual acuity and corneal tomography) and systemic characteristics and to describe an appropriate clinical algorithm for investigation and management of KC in this setting. METHODS: This was the retrospective cohort study of patients with ID (Down syndrome, autism, and other) in the cornea department of a tertiary referral ophthalmic hospital in Dublin, Ireland. Retrospective chart review was conducted on people with ID undergoing examination under anesthesia or crosslinking under general anesthetic. Key outcome data included corneal examination findings, corneal tomography, visual acuity, and examination findings (eg, type of ID, general anesthetic, and cardiac status). RESULTS: Mean age of the 24 patients was 31.9 years (66.7% male). ID type was Down syndrome (66.7%), autism (25%), and other (8.3%). KC was diagnosed in 98% of eyes, with 45.8% having untreatable advanced disease (57.1% of these bilateral), 39.6% amenable to corneal collagen crosslinking (35.7% of these bilateral), and 6.3% having corneal transplantation. Congenital heart defects were present in 37.5% of the Down syndrome group. There were no serious ocular or systemic adverse events. CONCLUSIONS: KC is strikingly prevalent in the ID population. Ireland has the highest rate of Down syndrome in Europe (26.3:10,000 live births). This group is rarely suitable for corneal transplantation, and corneal collagen crosslinking is an effective intervention to prevent progression to advanced KC in this already socially restricted group. We propose an algorithm for investigation/treatment and also recommend uniform pediatric KC screening/treatment in ID populations.

5-year longitudinal study of clinical and patient-reported outcomes in acute anterior uveitis.

INTRODUCTION: Acute anterior uveitis (AAU) is a frequently encountered form of uveitis, most commonly an immune-mediated condition associated with the HLA-B27 gene with or without spondyloarthritis, or idiopathic in nature. This study's aim was to measure clinical and patient-reported outcomes 5 years after the first episode of immune-mediated AAU. METHODS: This is a longitudinal observational study. Ninety-six patients who underwent evaluation at the time of presentation with their first episode of AAU were invited to return for evaluation 5 years later. Standardised ocular history, clinical examination and quality of life (QOL) assessment with the Short Form 36 (SF-36) and the Vision Core Measure 1 (VCM 1) questionnaire were completed and analysed. RESULTS: Fifty-four patients (56%) returned for subsequent assessment. Physical function was the only sub scale domain of the SF-36 that had significantly deteriorated over the 5 years since the first episode of AAU (45.95 vs. 49.37, p = 0.003). Only 7.4% (n = 4) of patients expressed "more than a little concern" regarding their vision, reflected by a VCM1 score of 2.0 or more. At 5 years, the mean best corrected visual acuity (BCVA) of eyes affected by AAU was LogMAR 0.02 and only 3% (n = 2) of eyes had a BCVA of less than Logmar of 0.3. Five affected eyes (8%) had developed cataract and no patients had developed glaucoma by the 5 year review. CONCLUSIONS: This study demonstrates that immune-mediated AAU has an excellent 5 year prognosis with minimal impact on patients' health and vision-related quality of life.