Negative effect of on-flare status on health utility index score assessed by the Short-Form Six-Dimension (SF-6D) was significant only for crFMF patients. Furthermore, the Sheehan Disability Score (SDSv3) showing the on-flare status resulted in significant functional impairment in all 3 disease cohorts through assessment of impact on work/school, social and family life. crFMF, MKD/HIDS and TRAPS negatively affected the quality of life (QoL) of adult and paediatric patients, including their physical, mental, psychosocial health, and social functioning. There remains, however, a high number of unmet needs for these patients to reduce their disease burden.crFMF, MKD/HIDS and TRAPS negatively affected the quality of life (QoL) of adult and paediatric patients, including their physical, mental, psychosocial health, and social functioning. There remains, however, a high number of unmet needs for these patients to reduce their disease burden. To characterise autoimmune congenital heart block (CHB) associated with a maternal diagnosis of primary Sjögren's syndrome (pSS) confirmed either before, concomitant or after the first pregnancy complicated with CHB. The following inclusion criteria were applied (i) Mothers with positive Ro/La autoantibodies detected previously or at the time of diagnosis of the first case of CHB; (ii) diagnosis of CHB confirmed by fetal echocardiography; (iii) AV block diagnosed in uterus, at birth or within the neonatal period (0-27 days after birth) (8); (iv) absence of anatomical cardiac abnormalities which might be causal of AV block; and (v) maternal fulfillment of the 2002 SS criteria before, during or after having a pregnancy complicated with CHB. We identified 49 cases of autoimmune CHB in children born from 44 mothers who had a mean age at the time of pregnancy of 30.3 years (range 18 to 41). At the time of diagnosis of autoimmune CHB, all mothers had positive anti-Ro antibodies and 28/44 (64%) were positive f confirmed pSS before having the first index case of CHB. In pSS, autoimmune CHB could be one of the first "indirect" signs of the disease in women of childbearing-age, in whom the diagnosis is confirmed several years later. check details Some maternal characteristics could be related with recurrent CHB, such as having an already-confirmed diagnosis of pSS and carrying the two Ro/La autoantibodies.In pSS, autoimmune CHB could be one of the first "indirect" signs of the disease in women of childbearing-age, in whom the diagnosis is confirmed several years later. Some maternal characteristics could be related with recurrent CHB, such as having an already-confirmed diagnosis of pSS and carrying the two Ro/La autoantibodies. To assess whether the use of digital image analysis (DIA) in primary Sjögren's syndrome (pSS) for the calculation of the total area of the salivary gland (SG), focus score (FS) and SG area occupied by the inflammatory infiltrate (area fraction, AF), was able to generate reproducible readings among different raters, reducing disagreement. Haematoxylin and Eosin digital slides from pSS and non-specific chronic sialadenitis (NSCS) patients were analysed blindly by 4 independent raters among 3 centres. Using an open-source software (QuPath) raters were asked to provide the total area of the gland i) using a grid-based method and ii) a software-based area-calculation tool, iii) the number of inflammatory foci and iv) the total area of the inflammatory infiltrate. Collected data was used to calculate the inter-rater agreement. For the calculation of the total SG area, DIA generated higher agreement among raters than grid-based calculation (inter-class correlation coefficient ICC=0.85 vs 0.98). Agreement for calculated total area of the inflammatory infiltrate (ICC=0.94) and for AF (ICC=0.94) was higher than infiltrates count number (ICC=0.54) and FS (ICC=0.56). AF achieved a 30% improvement over the FS at generating consensus among raters when used as a diagnostic cut-off. A digital approach achieved a far superior inter-rater agreement when calculating the total area compared to a grid-based approach. The calculation of AF proved superior to FS in correctly classifying pSS vs NSCS biopsies. We suggest that digitally calculated AF should be used alongside FS for large multi-centre studies to improve data harmonisation.A digital approach achieved a far superior inter-rater agreement when calculating the total area compared to a grid-based approach. The calculation of AF proved superior to FS in correctly classifying pSS vs NSCS biopsies. We suggest that digitally calculated AF should be used alongside FS for large multi-centre studies to improve data harmonisation. Rheumatoid arthritis (RA) patients with Sjögren's syndrome (SS) often present with more severe synovitis. We intended to clarify the impact of overlapping SS on ultrasound remission, functional ability and clinical decision-making in RA patients in a real-world cohort from 2009 to 2019. The medical records of RA patients in our medical centre from 2009 to 2019 were reviewed. Cox proportional hazards models of ultrasound remission and no disability (by health assessment questionnaire [HAQ]) were conducted in both the 1-to-1 nearest propensity score matched (PSM) and unmatched cohorts between RA patients with SS (RA-SS) and without (RA-noSS) to correct critical confounders. Four kinds of PSM methods were used and the corresponding average treatment effect on the treated (ATT) was calculated to clarify the effect of overlapping SS on distinguishable characteristics or drug prescription in RA patients. A total of 1100 RA patients were included in the study, of which 133 (12.1%) overlapped with SS. Among 256 patients consisting of 128 RA-SS and 128 RA-noSS after 1-to-1 nearest PSM, overlapping SS was associated with a 44%, 32% lower probability of reaching ultrasound remission, no disability in RA patients, respectively. More hydroxychloroquine (HCQ) usage, less biologic disease-modifying anti-rheumatic drugs (bDMARDs) prescription were confirmed to be correlated with overlapping SS by the robust PSM. Overlapping SS is associated with a lower probability of reaching ultrasound remission and no disability in RA patients. HCQ may still be the mainstream of clinical decision making in RA-SS patients.Overlapping SS is associated with a lower probability of reaching ultrasound remission and no disability in RA patients. HCQ may still be the mainstream of clinical decision making in RA-SS patients.