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Background: Juvenile Sjögren's syndrome (jSjS) is a rare autoimmune disease, primarily marked by the involvement of exocrine glands and a range of systemic symptoms, including small vessel vasculitis and Raynaud's phenomenon (RP). Objectives: Our study focused on investigating the microvascular status in jSjS patients by nailfold videocapillaroscopy (NVC) correlating it with clinical and serological features. Methods: Clinical information was gathered from thirteen consecutive jSjS patients (11 females and 2 males). The average age was 16 ± 4 years and participants were diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjS 1. Thirteen healthy controls (HCs) were age- and sex-matched. Clinical, laboratory, and instrumental data were collected, together with NVC examination. According to recently published standardised definitions in capillaroscopy, non-specific and specific NVC parameters were investigated 2. These included 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: RP was present in 8% of jSjS patients (Table 1). No specific NVC pattern for jSjS was identified, whereas the occurrence of abnormal capillary shapes were significantly greater in jSjS patients compared with HCs (p = 0.005, Figure 1). The reduction of capillary density correlated significantly with articular involvement, in particularly with arthralgias (p = 0.024). Microhaemorrhages correlated with lower C3 serum concentrations (p = 0.034). NVC abnormalities were not associated with SjS-specific instrumental tests (biopsy findings, imaging, Schirmer's test). Conclusion: 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 jSjS patients. REFERENCES: 1 Shiboski et al. Arthritis Rheumatol 2018. 2 Smith V et al. Aut Rev 2020. Acknowledgements: NIL. Disclosure of Interests: None declared.
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