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Introduction: Juvenile Sjögren's disease (jSjD) is a rare autoimmune disease characterized by exocrine gland involvement and systemic manifestations, including small vessel vasculitis and Raynaud's phenomenon (RP). We aimed to investigate the microvascular status in jSjD patients by nailfold videocapillaroscopy (NVC) and the potential correlations with clinical and serological features. Methods: Clinical data from thirteen consecutive jSjD patients (11 females and 2 males), with a mean age of 16 ± 4 years, diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjD, were collected including age- and sex-matched healthy controls (HCs). Clinical, laboratory, and instrumental data were collected, together with NVC examination. Non-specific and specific NVC parameters were investigated, such as capillary density, capillary dilations, giant capillaries, microhaemorrhages and abnormal shapes. Associations between NVC findings and clinical/serological features were explored and analysed using parametrical and non-parametrical tests. Results: Capillary density reduction correlated significantly with articular involvement (arthralgias) (p = 0.024). Microhaemorrhages correlated with lower C3 levels (p = 0.034). No specific NVC pattern for jSjD was identified, whereas abnormal capillary shapes were significantly higher in jSjD patients than HCs (p = 0.005). NVC abnormalities were not associated with SjD-specific instrumental tests (biopsy, imaging, Schirmer's test). RP was present in 8% of jSjD patients. Conclusions: The reduction of capillary density, as well as microhaemorrhages at NVC analysis, are significantly associated with some clinical aspects like articular involvement and serum biomarkers (C3 reduction). The NVC is suggested as safe and further analysis in jSjD patients. Key Points • Juvenile Sjögren's disease (jSjD) exhibits a higher rate of abnormal shapes at nailfold videocapillaroscopy (NVC) compared to matched healthy controls. • Articular involvement in jSjD is significantly associated with a lower capillary number at NVC. • Microhaemorrhages on NVC are associated with lower C3 levels, prompting this finding as a putative novel negative biomarker/prognostic factor for jSjD. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to analyze the risk factors for primary Sjögren's Syndrome (pSS) by conducting a meta-analysis of observational studies. Methods: Four electronic databases were searched from inception to August 2022. The search strategy included medical subject headings (MeSH) and text words. Outcomes were calculated and reported as the odds ratio (OR) and 95% confidence interval (CI). Results: Twelve studies consisting of nine case–control and three cohort studies were analyzed. Significant positive relationships between infection, a family history of autoimmune disease in first-degree relatives, negative stressful life events, CGGGG insertion/deletion polymorphisms in the IRF5 gene and the onset of pSS were found, with pooled ORs and 95% CIs of 2.73 (1.93, 3.86), 5.93 (3.34, 10.52), 1.69 (1.27, 2.24) and 2.69 (1.97, 3.66), respectively. In contrast, the results showed that a history of smoking was not associated with the onset of pSS, with a pooled OR and 95% CI of 1.39 (0.76, 2.53). However, a statistically significant negative association between current smoking and pSS was detected, with a pooled OR and 95% CI of 0.4 (0.29, 0.83). Conclusions: Our research indicated that infection, a family history of autoimmune disease in first-degree relatives, negative stressful life events and CGGGG insertion/deletion polymorphisms in the IRF5 gene might be risk factors for pSS. In contrast, our study demonstrated that a history of smoking was not associated with the onset of pSS, whereas current smoking was negatively associated with pSS onset. Systematic review registration: We registered this review on INPLASY (https://inplasy.com/) under registration number INPLASY202230005. [ABSTRACT FROM AUTHOR]

To gain insight into the Sjögren's disease (SjD) patient experience using a survey generated by patients and providers. We evaluated the results of the 2016 Sjögren's Foundation survey, with 25 questions designed in a collaborative effort between the Foundation, patients with SjD, SjD provider experts, and a marketing research company. We used descriptive statistics to provide a thorough understanding of SjD demographics, symptoms, quality of life (QoL), cost, and treatments. Analyses revealed high symptoms, QoL, and financial burdens in SjD. Dry mouth and eye were the most commonly reported symptoms (94 and 93%, respectively). The most frequent extra-glandular symptoms included fatigue, dry or itchy skin, and morning stiffness. The top three aspects of QoL most impaired included (i) sex life (53%), (ii) participating in hobbies/social activities/extracurricular activities (52%), and (iii) job/career or ability to work (49%). SjD respondents commonly reported taking health food supplements/remedies, vitamin D, and exercising, in addition to taking treatments for symptomatic dryness. SjD costs were high, including a total yearly cost, on average, of $2026 for dental care. SjD respondents reported that dryness and risk factors for lymphoma and fatigue are essential to address with new therapies. In this comprehensive overview of the SjD experience, we demonstrated a high burden of disease to SjD respondents, including symptoms, QoL, and financial burden. We also identify the top goals of therapy for new systemic SjD therapies. Key Points • The top three symptoms or signs that patients with Sjögren's hope new treatments will address are dryness, fatigue, and reduction in lymphoma or blood cancer risk • The top aspects of quality of life reported to be impaired by Sjögren's are sex life, hobbies, social activities and extracurricular activities, job/career or ability to work, and finding the correct word during conversations • Patients with Sjögren's have a yearly mean dental cost of $2026 but also have high costs associated with prescription medications, healthcare appointments, over-the-counter medications, alternative therapies, and medical equipment [ABSTRACT FROM AUTHOR]

Objective: To investigate the value of 68 Ga-PSMA-11 positron emission tomography/computerized tomography (PET/CT) in evaluating lacrimal and salivary glands function. Methods: Ten patients with pSS and 18 healthy volunteers were recruited in this study. All participants underwent 68 Ga-PSMA-11 PET/CT, and the patients with pSS performed salivary gland scintigraphy the next day. The maximum standardized uptake value (SUVmax), average of the standard uptake value (SUVavg), the average CT value (CTavg), and volume (V) in the region of interest (ROI) of each lacrimal and salivary gland were analyzed in68Ga-PSMA-11 PET/CT. The uptake ratio (UR) of the bilateral parotid gland and submandibular gland was calculated in salivary gland scintigraphy (SGS). Statistical analysis was processed by the SPSS software and the MedCalc software. A p-value of < 0.05 was considered as statistically significant. Results: Almost all the parameters of pSS were significantly lower than those of the control group (p < 0.05). The left parotid gland (PG) UR was positive correlation with left PG SUVmax (r = 0.758, p = 0.011) and left PG SUVavg (r = 0.770, p = 0.009); the right PGUR was positive correlation with right PG SUVmax (r = 0.721, p = 0.019) and right PG SUVavg (r = 0.721, p = 0.019). The SUVmax and SUVavg of both sides of acrimal and salivary glands had area under the receiver operating curve values greater than 0.5. Conclusions: 68 Ga-PSMA-11 PET/CT can simultaneously enable the visualization of lacrimal glands and salivary glands and be used to evaluate the lacrimal and salivary glands function. Key Points • We have firstly investigated the value of 68 Ga-PSMA-11 PET/CT in evaluating lacrimal and salivary glands function in patients with pSS 68 Ga-PSMA-11 PET/CT can simultaneously allow the visualization of lacrimal glands and salivary glands. • The results of the present study imply that 68 Ga-PSMA-11 PET/CT can be used to evaluate the lacrimal and salivary glands function in patients with pSS meanwhile. [ABSTRACT FROM AUTHOR]

The objective of this review was to assess and evaluate whether the published diagnostic accuracy studies provide evidence to sustain the current diagnostic guidelines put forth by ACR/EULAR used for patients with suspected Sjögren's syndrome (SS). Literature databases, including Medline, Embase, and EBM Reviews, were searched for relevant studies on the correlation between ACR/EULAR criteria, particularly those with a direct comparison between their accuracy in diagnosing Sjögren's syndrome. We followed Cochrane, QUADAS-2, and STARD guidelines and the four-phase flow diagram by the PRISMA Statement. Reports in several languages, but only human studies were considered. Three studies assessed the accuracy of the current diagnostic tests, and these did not present adequate designs that would allow a well-supported conclusion with a high level of certainty. Due to significant clinical and methodological heterogeneity, a meta-analysis was not performed. A qualitative review of the papers was undertaken. Neither the comparative nor the non-comparative study designs permit conclusive recommendations regarding an alternative diagnostic pathway for SS. Well-designed studies of the diagnostic accuracy of SS tests are needed to validate current guidelines or to suggest changes to the current guidelines. [ABSTRACT FROM AUTHOR]