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.

Advancing Our Understanding of Corneal Herpes Simplex Virus-1 Immune Evasion Mechanisms and Future Therapeutics.

Herpes stromal keratitis (HSK) is a disease that commonly affects the cornea and external eye and is caused by Herpes Simplex Virus type 1 (HSV-1). This virus infects approximately 66% of people worldwide; however, only a small portion of these people will develop symptoms in their lifetime. There is no cure or vaccine available for HSV-1; however, there are treatments available that aim to control the inflammation caused by the virus and prevent its recurrence. While these treatments are beneficial to those suffering with HSK, there is a need for more effective treatments to minimise the need for topical steroids, which can have harmful effects, and to prevent bouts of disease reactivation, which can lead to progressive corneal scarring and visual impairment. This review details the current understanding of HSV-1 infection and discusses potential novel treatment options including microRNAs, TLRs, mAbs, and aptamers.

Investigation of type I interferon responses in ANCA-associated vasculitis.

Type I interferon (IFN) dysregulation is a major contributory factor in the development of several autoimmune diseases, termed type I interferonopathies, and is thought to be the pathogenic link with chronic inflammation in these conditions. Anti-neutrophil cytoplasmic antibody (ANCA)-Associated Vasculitis (AAV) is an autoimmune disease characterised by necrotising inflammation of small blood vessels. The underlying biology of AAV is not well understood, however several studies have noted abnormalities in type I IFN responses. We hypothesised that type I IFN responses are systemically dysregulated in AAV, consistent with features of a type I interferonopathy. To investigate this, we measured the expression of seven interferon regulated genes (IRGs) (ISG15, SIGLEC1, STAT1, RSAD2, IFI27, IFI44L and IFIT1) in peripheral blood samples, as well as three type I IFN regulated proteins (CXCL10, MCP-1 and CCL19) in serum samples from AAV patients, healthy controls and disease controls. We found no difference in type I IFN regulated gene or protein expression between AAV patients and healthy controls. Furthermore, IRG and IFN regulated protein expression did not correlate with clinical measurements of disease activity in AAV patients. Thus, we conclude that systemic type I IFN responses are not key drivers of AAV pathogenesis and AAV should not be considered a type I interferonopathy.

Changing trends in corneal transplantation: a national review of current practices in the Republic of Ireland.

BACKGROUND: First Irish National Corneal Transplant Registry report. AIM: To report about current corneal transplantation practices in Ireland including patient demographics, indications and types of transplant performed and to compare the findings with other developed countries. METHODS: Nationwide retrospective review of the corneal transplants performed in Ireland between 2016 and 2019. RESULTS: Overall, 536 keratoplasties were carried out: 256 (47.8%) Penetrating Keratoplasties (PK), 212 (39.6%) Descemet Stripping Automated Endothelial Keratoplasties (DSAEK), 30 (5.6%) Descemet Membrane Endothelial Keratoplasties (DMEK), and 25 (4.7%) Deep Anterior Lamellar Keratoplasties (DALK). The most common indication was Keratoconus (KC, 19%), followed by Fuchs endothelial dystrophy (FED, 18.8%), and Pseudophakic bullous keratopathy (PBK, 17%). KC (34%) and re-grafting (17%) were the leading indications for PK, whereas FED and PBK were the major indications for DSAEK (38% and 33%) and DMEK (67% and 20%), respectively. During the period studied, the number of transplants increased from 11.3 to 14 grafts per month. The number of PKs remained stable, whereas Endothelial Keratoplasties, DSAEK and DMEK, increased (3.8 to 5.6 and 0.2 to 1.6 per month, respectively), becoming the most commonly performed grafts since 2018. Only a small number of DALK were performed. CONCLUSIONS: Corneal transplantation in Ireland is following international trends as endothelial procedures have become the most common approach since 2018. However, a low overall number of transplants is performed in Ireland compared with other countries suggesting that care pathways should be implemented to improve access to corneal transplantation.

Refractory recurrent ocular graft versus host disease.

Ocular graft-versus-host disease (GVHD) is one of the most frequent and long-term complications affecting patients after haematopoietic stem cell transplantation. It is associated with significant morbidity and a marked reduction in quality of life. Although common, currently there are no widely accepted guidelines available for its management, and no suggested regime of treatment that is completely satisfactory. So far, prophylactic treatment strategies for ocular GVHD have yet to be developed and treatment is normally initiated based on symptoms often after permanent ocular tissue changes and surface damage has occurred. Here we describe a case of recurrent ocular GVHD and its associated complications that was highly refractory to treatment.

Corneal melt secondary to eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis that can affect any organ system. It is most often characterised by chronic airway inflammation along with prominent peripheral blood eosinophilia, although the disease can affect the cardiovascular, gastrointestinal, renal or central nervous systems. Ocular manifestations are uncommon and when they do occur, are varied in their clinical presentations. To the best of our knowledge, this is the first case of corneal melt secondary to EGPA to have been reported.

The effect of geodemographic factors on the attendance rates at a regional diabetic retinopathy treatment centre.

AIM: To determine whether geodemographic factors, specifically age, gender or commuting distance, affect the attendance rates of patients referred to a Diabetic Retinopathy Treatment (DRT) Centre from the Irish National Diabetic Retinal Screening (DRS) Programme. METHODS: A retrospective analysis of the first 1200 patients who were referred for ophthalmic assessment between November 2013 and June 2015 to Cork University Hospital's (CUH) DRT clinic from the DRS was completed. RESULTS: A total of 972 (81%) patients attended all their scheduled DRT appointments. The attendance rates for those living in counties Cork and Kerry were 83.9% and 72.5%, respectively. Fifty-seven (4.75%) patients failed to attend any of their clinic appointments (2.2% of Cork and 11.8% of Kerry referrals). Patients living within 60 km of the treatment centre were more likely to attend (p = 0.013). Both age and gender were also shown to have a statistically significant impact on attendance, with those under 40 years of age (p < 0.001) and male patients (p = 0.02) less likely to attend their scheduled appointments. CONCLUSION: This study suggests a correlation between geodemographic factors (age, gender and commuting distance) and attendance at a DRT Centre. The longer commuting distance for Kerry patients contributes to their higher non-attendance rates indicating a need to provide a Kerry-based satellite treatment centre to improve accessibility.