Quality of life questionnaires validate a remote approach to ophthalmic management of primary Sjögren's syndrome.

Those with underlying autoimmune conditions were met with unparalleled challenges and were disproportionately affected by the COVID-19 pandemic. As such, we aimed to measure the impact of the pandemic on symptoms and the health and vision related quality of life (HR-QoL, VR-QoL) in patients with Primary Sjögren's Syndrome (pSS). Nineteen (55.9%) participants returned questionnaires for analysis, (17 female and 2 male, 61.6 years ± 9.9). There was no significant change in participants HR-QoL or VR-QoL, indicating that those with pSS remained resilient with regard to their physical and mental health throughout the pandemic. Furthermore, QoL was maintained despite 73.7% of participants having had outpatient appointments cancelled, delayed or rescheduled. Participants reported a lower QoL and feeling tenser in the COV19-QoL (3.3 ± 1.4 and 3.2 ± 1.3) representing feelings of apprehension and stress felt amongst the general population since the pandemic. Overall, and in spite of the concern caused by the COVID-19 pandemic for patients with autoimmune diseases, the health and well-being of patients with pSS remained stable. These findings strongly support the use of validated HR and VR-QoL questionnaires as an adjunct to the telemedicine consultation when assessing patients with pSS, offering an alternative to face-to-face consultations in post-pandemic era.

Severity of COVID-19 infection in primary Sjögren's syndrome and the emerging evidence of COVID-19-induced xerostomia.

Since the beginning of the COVID-19 disease pandemic caused by the new coronavirus SARS-CoV-2, the disease has claimed over 205M cases (205,338,159) and 4,333,094 deaths (WHO dashboard - accessed 15/08/2021). In addition to the overwhelming impact on healthcare systems treating acutely ill patients, the pandemic has had an impact on all other aspects of health care delivery, including the management of chronic diseases, the risk that is posed in patients with chronic conditions and the risk of the infection itself in those with chronic conditions. Autoimmune rheumatic diseases (ARDs), including primary Sjögren's syndrome (pSS), characterised by immune dysregulation affecting several organs in variable severity, have been of particular interest given the accelerated phase of the immune response in the course of SARS-CoV-2 infection leading to the acute inflammatory response and respiratory distress syndrome or multi-organ failure. On the other hand, the effect of immunosuppressive drug therapies can represent a double edge sword on the course of the disease, either by increased susceptibility to and severity of the infection, or by preventing the accelerated inflammatory response induced by the infection. Additionally, the long-term impact of SARS-CoV-2 infection on the host immune system has led to the onset of novel complex clinical manifestations, comprised under the large umbrella of "long-COVID", which we are only starting to understand. In this review we focus on two interrelated aspects: i) the impact of COVID-19 on patients with pSS and ii) the emerging evidence of long-term xerostomia after SARS-CoV-2 infection.

Post-COVID-19 syndrome in patients with primary Sjögren's syndrome after acute SARS-CoV-2 infection.

OBJECTIVES: To analyse the frequency and characteristics of post-COVID-19 syndrome in patients with primary Sjögren's syndrome (pSS) affected by acute SARS-CoV-2 infection. METHODS: By the first week of April 2021, all centres included in the Big Data Sjögren Consortium were contacted asking for patients included in the Registry diagnosed with SARSCoV-2 infection according to the ECDC guidelines. According to the NICE definitions, symptoms related to COVID-19 were classified as acute COVID-19 (signs and symptoms for up to 4 weeks), ongoing symptomatic COVID-19 (presence of signs and symptoms from 4 to 12 weeks) and post-COVID-19 syndrome (signs and symptoms that continue for > 12 weeks not explained by an alternative diagnosis after a protocolized study). RESULTS: We identified 132 patients who were followed a mean follow-up of 137.8 days (ranging from 5 days to 388 days) after being diagnosed with COVID-19. In the last visit, 75 (57%) patients remained symptomatic: 68 (52%) remained symptomatic for more than 4 weeks fulfilling the NICE definition for ongoing symptomatic post-COVID-19, and 38 (29%) remained symptomatic for more than 12 weeks fulfilling the definition of post-COVID-19 syndrome. More than 40% of pSS patients reported the persistence of four symptoms or more, including anxiety/depression (59%), arthralgias (56%), sleep disorder (44%), fatigue (40%), anosmia (34%) and myalgias (32%). Age-sex adjusted multivariate analysis identified raised LDH levels (OR 10.36), raised CRP levels (OR 7.33), use of hydroxychloroquine (OR 3.51) and antiviral agents (OR 3.38), hospital admission (OR 8.29), mean length of hospital admission (OR 1.1) and requirement of supplemental oxygen (OR 6.94) as factors associated with a higher risk of developing post-COVID-19 syndrome. A sensitivity analysis including hospital admission in the adjusted model confirmed raised CRP levels (OR 8.6, 95% CI 1.33-104.44) and use of hydroxychloroquine (OR 2.52, 95% CI 1.00-6.47) as the key independent factors associated with an enhanced risk of developing post-COVID-19 syndrome. CONCLUSIONS: This is the first study that analyses the frequency and characteristics of post-COVID-19 syndrome in patients affected by a systemic autoimmune disease. We found that 57% of patients with pSS affected by COVID-19 remain symptomatic after a mean follow-up of 5 months. The risk of developing post-COVID-19 syndrome in patients who required hospitalisation was 8-times higher than in non-hospitalised patients, with baseline raised CRP levels and the use of hydroxychloroquine being independent risk factors for post-COVID-19.

Sjögren-related cardiomyopathy presenting with cardiogenic shock.

Previous reports have described non-ischaemic cardiomyopathy related to a variety of autoimmune diseases. However, very few case reports describe Sjögren disease as a contributing factor to cardiomyopathy. We report the case of a 69-year-old woman with a history of Sjögren disease who presented with cardiogenic shock. Laboratory testing and cardiac MRI revealing apical septal late gadolinium enhancement were consistent with an autoimmune aetiology. After ruling out ischaemic, infectious and other possible causes, the patient's clinical presentation was thought to be related to underlying Sjögren disease. She was treated with intravenous steroids and evidence-based heart failure therapy, but she eventually died after having declined heart transplantation. Given the rarity of Sjögren disease, no diagnostic criteria or standard treatment has been established for cardiomyopathy related to this disease. Diagnosis should be considered in patients who show evidence of autoimmune processes after other possible causes are ruled out.