Background: The recently published 2019 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria for systemic lupus erythematosus (SLE) were developed to increase the reliability and identification of SLE, especially in early disease. With the emergence of several new drugs for SLE, identifying and treating patients early are more important than ever. Methods: Data of 446 SLE patients evaluated in our center between 1996–2019 and 226 controls with other autoimmune diseases evaluated between 2001–2022 were retrospectively analyzed. The sensitivity and specificity of the 2019 ACR/EULAR criteria were compared to the 2012 SLICC and the 1997 ACR criteria. Results: The 2019 ACR/EULAR criteria showed very good sensitivity (86.6%) compared to the 1997 ACR criteria (76.7%), p < 0.001, with a trend toward significance compared to the 2012 SLICC criteria (83.6%), p = 0.072. Their sensitivity remained high (87.6%) in patients with a short disease duration. The specificity of the 2019 ACR/EULAR criteria (91.2%) was statistically lower than the 2012 SLICC (96.0%) and 1997 ACR criteria (95.1%), p = 0.007 and p = 0.012, respectively, but still had a very high value. A total of 22 controls (9.7%) fulfilled at least one set of criteria (15 patients with MCTD, 5 with UCTD, and 2 with SSc). Conclusion: In this large real-life cohort, the 2019 ACR/EULAR criteria had very good performance compared to the 2012 SLICC and 1997 ACR criteria.

Background/Objectives: Systemic sclerosis (SSc) is a multisystemic chronic autoimmune disease, which leads to disability and possibly early retirement. The objective of our study was to explore the associations between employment status (ES) and demographic, clinical and functional features in a single-center EUSTAR cohort. Methods: Consecutive patients with SSc examined between November 2011 and June 2023, who were under the age of retirement in our country (62 years for women, 65 for men at the time), were included. All patients underwent a comprehensive clinical assessment and filled in a work assessment questionnaire as well as two validated health-related questionnaires: the Scleroderma Health Assessment Questionnaire (SHAQ) and the Duruoz Hand Index (DHI). Associations between ES and potential predictors (education level, disease characteristics, work conditions, SHAQ and DHI) were tested using logistic regression adjusted for age and gender. Results: Ninety-one patients (mean ± SD age 53.7 ± 11.8 years, twenty-two with diffuse skin involvement, fifty-six with a history of digital of digital ulcers (DUs)), were included. Only 22 patients were still employed, while 69 were retired, of which 38 retired because of SSc. Among the employed, nine performed manual labor, nine spent many hours standing and three had to work in a cold environment. When potential predictors were tested separately, adjusted for age and sex, patients with higher education (OR (95% CI) 11.36 (2.03–63.36), p = 0.006) and no history of digital ulcers had higher odds of being employed. The presence of joint contractures and weightlifting as a work demand were associated with unemployment. In a multivariable model, higher education (OR 5.91, 95% CI 0.97–36.09, p = 0.054 and younger age (OR 0.90, 95% CI 0.85–0.96, p = 0.001) were independently associated with continued employment. High school education did not show a significant effect (OR 0.089, 95% CI 0.015–0.530, p = 0.008). Patients with a history of digital ulcers had the lowest employment rates compared to those with no digital ulcer history. No significant associations were found between employment status and SHAQ or DHI scores. Conclusions: SSc is associated with significant work disability and early retirement. Higher education, the lack of Dus and younger age were highly associated with staying employed. Given the rarity of SSc, we consider that our good sample size (n = 91) reflects disease prevalence, but results should be tested in other studies and the single center should be considered when interpreting generalizability.

Heart rate variability is a valuable non-invasive marker of autonomic nervous system function, reflecting the balance between the sympathetic and parasympathetic systems. Rheumatic diseases are characterised by systemic inflammation, autoimmune activity, and sometimes direct involvement of the cardiovascular system. These factors can influence autonomic nervous system function, thereby affecting heart rate variability. There is a growing acknowledgement of reduced Heart rate variability (HRV) in rheumatic diseases, which is associated with systemic inflammation, autonomic dysfunction, and an increased cardiovascular risk. Despite its clinical relevance, autonomic dysfunction remains under-recognised in rheumatology. This chapter reviews the current evidence regarding HRV alterations in rheumatic diseases and highlights its utility in disease monitoring, risk assessment, and potential therapeutic modulation.