Objective: This study evaluates assessment and prescription trends in systemic sclerosis across different Indian healthcare settings, with a focus on diagnostic practices such as screening for interstitial lung disease, pulmonary hypertension, and adherence to recommended treatment protocols. The goal is to identify disparities and areas for improvement in the management of systemic sclerosis. Methods: A cross-sectional questionnaire-based survey was conducted among rheumatologists from teaching and non-teaching hospitals across India. Data collection focused on key diagnostic practices such as the modified Rodnan skin score, chest imaging, pulmonary function tests, and echocardiography. Organ-specific prescription trends were collected and compared between teaching and non-teaching centres. Results: The response rate for the survey was 70.5%. Teaching centres demonstrated higher adherence to performing modified Rodnan skin score at baseline (72.2%) compared to non-teaching hospitals (38.4%). For interstitial lung disease screening, overall, 93.7% performed chest imaging, with only 31.4% utilizing a High-Resolution CT thorax as the screening tool. Teaching centres performed 6MWT (79.5%) more often than non-teaching centres (64.7%). Echocardiography was commonly used for screening pulmonary hypertension (96.4%), while 16.5% reported using right heart catheterization. Steroids were used by 79.9% of participants at low doses (<10 mg) for a duration of less than 3 months, commonly for myositis(68%). Methotrexate(49.8%) and mycophenolate (38.3%) were the most prescribed first-line agents for systemic sclerosis-skin involvement. For systemic sclerosis-interstitial lung disease, mycophenolate (95%) was the most commonly used immunosuppression. Sequential addition of antifibrotic(62.4%) to immunosuppression was preferred over an upfront combination in systemic sclerosis-interstitial lung disease. The majority treated uncomplicated Raynaud's phenomenon with calcium channel blockers, followed by PDE5 inhibitors (61.4%). An upfront combination of endothelin receptor antagonists and PDE5i for systemic sclerosis-pulmonary hypertension was reported by 42.2%. Conclusion: The study highlights differences in systemic sclerosis management trends among Indian rheumatologists. Despite variations in disease-encounter and practice settings, adherence to international recommendations in key domains and areas for further improvement are brought to light.

Background: The impact of rheumatic diseases (RDs) on the Quality of Life (QoL) of antenatal women remains underexplored. This study aimed to investigate health-related QoL among pregnant and breastfeeding women with RDs in comparison to their healthy counterparts, utilizing data from the COVAD-2 e-survey database. Objectives: To assess health-related QoL using Patient-Reported Outcome Measurement Information System (PROMIS) instruments, focusing on Global Physical Health (GPH) and Global Mental Health (GMH) scores. Comparative analyses were conducted among four groups: 1) Non-pregnant/breastfeeding healthy, 2) Non-pregnant/breastfeeding RDs, 3) Pregnant/breastfeeding healthy, and 4) Pregnant/breastfeeding RDs. The study sought to identify factors influencing GPH and GMH scores within each group. Methods: Demographic information, diagnosis of RDs, comorbidities, disease activity, and treatments were analyzed using data from the COVAD-2-e-survey database. GPH, and GMH scores were compared among the four groups, and multivariable regression analysis was employed to identify the factors affecting GPH and GMH scores within each group. Results: After excluding missing values, our analysis encompassed 2931 cases across four groups: 1) Non-pregnant/breastfeeding healthy (1337 cases), 2) Non-pregnant/breastfeeding RDs (1459 cases), 3) Pregnant/breastfeeding healthy (77 cases), and 4) Pregnant/breastfeeding RDs (58 cases).The General Physical Health (GPH) median score peaked in the pregnant/breastfeeding healthy group (17 [10-20]), contrasting with the lowest score observed in Non-pregnant/breastfeeding RDs (14 [4-20]) (Figure 1). Among RD patients, pregnant/breastfeeding groups exhibited significantly higher GPH scores compared to non-pregnant/breastfeeding groups (p=0.01), an observation absent in healthy controls. Influencing factors in RDs included ethnicity, with Caucasians showing better scores than Asians (p=0.017), pregnant/breastfeeding status (p=0.002), concomitant comorbidities (p=0.041), and mental disorders (p=0.001).Similarly, the General Mental Health (GMH) median score was highest in the pregnant/breastfeeding healthy group (17 [4-20]) and lowest in Non-pregnant/breastfeeding RDs (15 [4-20]) (Figure 1). Pregnant/breastfeeding RDs groups exhibited significantly higher GMH scores than their non-pregnant/breastfeeding counterparts (p=0.003), a distinction not observed in healthy controls. Influencing factors in RDs included ethnicity, with Caucasians showing better scores than African Americans (p=0.000) or Asians (p=0.025), pregnant/breastfeeding status (p=0.001), and concomitant mental disorders (p=0.000). Conclusion: Women with RDs exhibit better mental and physical QoL during the antenatal period than their non-pregnant/breastfeeding counterparts—a distinction not observed in healthy controls, possibly as disease is planned in safe periods or goes into remission during antenatal period and this is reassuring. Ethnicity, comorbidities, and mental disorders have a substantial impact on QoL in this demographic, calling for targeted healthcare interventions that acknowledge the unique features of women of childbearing age with RDs. Further, these observations call for long term prospective international studies to comprehensively address the aspects of QoL in this specific demographic, enabling the development of more informed and personalized healthcare strategies. REFERENCES NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.Figure 1Comparison of Global Physical Health (GPH) scores and Global Mental Health (GMH) scores within four groups: 1) Non-pregnant/breastfeeding healthy, 2) Non-pregnant/breastfeeding RDs, 3) Pregnant/breastfeeding healthy, and 4) Pregnant/breastfeeding RDs.

Background: Glucocorticoids (GC) are commonly used in the management of systemic lupus erythematous (SLE). However, there are marked differences in GC use globally and this variation may be attributed to differences in clinical practices, regional preferences, local guidelines and a lack of consensus of standardised international recommendations. The global patterns of GC use and factors influencing these diverse practices are currently poorly understood. Objectives: We sought to evaluate the variations in GC use in the management of SLE globally and factors associated with patterns of GC therapy. Additionally, we aimed to identify factors associated with GC use based on patient demographics, disease activity, Human Development Index (HDI) and concomitant DMARDs use. Methods: Data for patients with SLE from more than 80 countries was extracted from the COVAD-2 database. Individuals were grouped into regions based on geographical location. A logistic regression model was constructed to identify associations between region and GC use. Multivariate adjustment was made for age, gender, health status, hydroxychloroquine (HCQ) use and HDI (a composite index formulated by the United Nations to rank countries into tiers of development). Odds ratios (OR) were recorded with corresponding 95% confidence intervals (CI). To explore regional differences, mean steroid use by region was mapped globally. Regions with fewer than five patients were excluded from mapping. Results: A total of 1292 SLE patients were included in the study. The majority of patients were from Asia (35.9%) and Europe (27.2%) with 94.9% being female. Median age was 40 years with a median disease duration of 8 years. Full patient demographics are summarised in Table 1. More than half of the patients (54.6%) were on GC with 13.2% being on high dose (defined as ≥20 mg Prednisolone equivalent/ day). Patients on GC were younger than those not on GC (median 38 years, IQR 30-48 vs 43 years, IQR 31-50, p <0.001) and were younger at age of diagnosis of SLE (median 26 years, IQR 20-34 vs 29 years, IQR 22-39, p<0.001). On a regional level, GC use was highest in Asia (64.4%) compared to South America (58.9%), Africa (58.6%), North America (48.3%) and Europe (41.5%). HCQ use was associated with reduced risk of being on GC [all GC unadjusted OR 0.65 (95% CI 0.51 - 0.83) showing the potential steroid sparing capacity of HCQ. Regional variability of GC use is summarised in Figure 1A, which shows low frequency of GC use noted in North Africa compared with high levels of GC use in sub-Saharan Africa. In the logistic regression model, region associated with GC use. Compared with Europe, patients in Asia, Africa and South America were more likely to use GC, with the highest odds of GC use seen in Central Africa and South Asia (OR 7.09 and 5.24, respectively). (Figure 1B). Conclusion: We identified substantial regional variations in GC use in the management of SLE globally. HCQ use associated with a reduced risk of GC exposure, indicating potential steroid-sparing effect. We demonstrate ongoing regional variability in GC use after controlling for patient demographics, HCQ use and HDI. It is possible there are additional factors influencing GC prescribing, such as prevalence, severity and phenotype of disease, which we have not been able to adjust for. Regional variation may reflect differences in availability, accessibility and affordability of other treatments including DMARDs and biologic therapies This variation may also represent variation in clinical guidelines, practices, and patient/physician preferences and beliefs in treatment of the disease. The study highlighted the need for more research and education on the optimal use of steroids in SLE patients internationally. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: Gagandeep Sukhija: None declared, Eman Elfar: None declared, Amelia Holloway: None declared, Katie Bechman: None declared, Sreoshy Saha: None declared, Tsvetelina Velikova: None declared, Elena Nikiphorou: None declared, Esha Kadam: None declared, Carlos Enrique Toro-Gutierrez: None declared, Rohit Aggarwal Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therepeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio., Vikas Agarwal: None declared, Latika Gupta: None declared, Chris Wincup: None declared.

Background: COVID-19 has been suggested as a possible trigger of disease flares in patients with rheumatoid arthritis (RA). However, factors associated with disease flares remain unknown. Objectives: This study aimed to identify factors associated with disease flares in patients with RA following COVID-19. Methods: We selected patients with RA from an online e-survey data from the COVID-19 vaccination in autoimmune diseases (COVAD) study. Demographic data, patient-reported outcomes, comorbidities and pharmacologic treatment were extracted from the database. Disease flare-ups were derived from the e-survey database. Factors associated with disease flare-ups were determined by multivariate logistic regression analysis. Results: In total, 1928 patients with RA were extracted from the COVAD database. Younger age (odds ratio (OR): 0.98, 95% CI: 0.96 – 0.99, p<0.001), ethnicity other than Asian, past history of tuberculosis (OR: 3.80, 95% CI: 1.12 – 12.94, p=0.033), treatment with methotrexate (OR: 2.55, 95% CI: 1.56 – 4.17, p<0.001), poor global physical health (OR: 1.07, 95% CI: 1.00 –1.15, p=0.044) and mental health (OR: 0.91, 95% CI: 0.87 –0.95, p<0.001) were independent factors associated disease flares in patients with RA. Table 1 represents factors associated with disease flare in patients with RA after COVID breakthrough infections and Figure 1 represents forest plots of symptoms of disease flare. Conclusion: Our study highlights important socio-demographic, clinical characteristics and mental health to be associated with flares in patients with RA. This finding may help determine relevant strategies to proactively manage RA patients at risk of flares. REFERENCES: NIL. Acknowledgements: Myositis Association, Myositis India, Myositis UK, Myositis Support and Understanding, the Myositis Global Network, Deutsche Gesellschaft für Muskelkranke e.V. (DGM), Dutch and Swedish Myositis patient support groups, Cure JM, Cure IBM, Sjögren’s India Foundation, Patients Engage, Scleroderma India, Lupus UK, Lupus Sweden, Emirates Arthritis Foundation, EULAR PARE, ArLAR research group, AAAA patient group, Myositis Association of Australia, APLAR myositis special interest group, Thai Rheumatism association, PANLAR, AFLAR NRAS, Anti-Synthetase Syndrome support group, and various other patient support groups and organizations. Disclosure of Interests: None declared.Figure 1Forest plots of symptoms of breakthrough infections and disease flare. Table 1Factors associated with disease flare in patients with RA after COVID breakthrough infectionsUnivariateMultivariateOR95% CIP valueOR95% CIP valueAge0.98(0.97-0.99)<0.001**0.98(0.96-0.99)<0.001**Gender FemaleReferenceMale0.76(0.46-1.24)0.268Ethnicity AsianReferenceReferenceCaucasian3.73(2.15-6.47)<0.001**4.33(2.43-7.72)<0.001**African American/ African4.13(2.02-8.46)<0.001**3.64(1.73-7.64)0.001**Hispanic3.45(1.78-6.66)<0.001**3.71(1.88-7.34)<0.001**Native American/Indigenous/Pacific Islander1.50(0.19-12.01)0.7041.52(0.18-12.60)0.697Mixed2.87(1.17-7.02)0.021*2.94(1.18-7.34)0.021*Other2.65(1.16-6.09)0.021*2.83(1.21-6.64)0.017*Comorbidities Asthma1.49(0.99-2.25)0.0571.21(0.78-1.90)0.394Interstitial Lung Disease1.89(0.99-3.58)0.0532.01(1.00-4.04)0.051Tuberculosis3.41(1.06-10.97)0.040*3.80(1.12-12.94)0.033*Medication Glucocorticoid1.98(1.20-3.25)0.007**1.26(0.71-2.23)0.425Methotrexate2.80(1.82-4.31)<0.001**2.55(1.56-4.17)<0.001**Hydroxychloroquine2.90(1.44-5.84)0.003**1.78(0.76-4.18)0.186Anti TNF agents#3.72(1.68-8.24)0.001**2.33(0.94-5.77)0.066JAK inhibitors +4.27(1.27-14.29)0.019*2.93(0.80-10.78)0.106PROMIS global physical health1.07(1.00-1.15)0.044*1.09(1.00-1.18)0.040*PROMIS global mental health0.91(0.87-0.95)<0.001**0.91(0.87-0.95)<0.001**By Logistic regression. *p<0.05, **p<0.01.#infliximab, adalimumab, certolizumab, golimumab, etanercept+tofacitinib, baricitinib, upadacitinib

Background: Idiopathic inflammatory myopathies (IIM) comprise a diverse range of multisystem diseases, with patients reporting varying levels of pain. Understanding the core drivers of pain is crucial for individualized decision making in treatment, as it is likely to have a significant impact on patient experience. Objectives: Our goal was to discern differences in pain perception among patients with autoimmune diseases, specifically identifying the factors associated with pain perception differences in IIM patients. Methods: Data were collected from the COVAD-2 e-survey, an international collaborative research study of 15,832 participants (of which 1046 had IIM). Demographic details, IIM subtype, clinical features, comorbidities, medications, and validated patient-reported outcome measures (PROMs) were collected. The IIM diagnoses were self-reported, but must have been made by a rheumatologist, dermatologist, or neurologist. The Fatigue 4A and PROMIS PF 10 scores were calculated based on validated questionnaires and used as functional outcomes.Respondents were classified into four groups: healthy (no autoimmune disease), IIM, systemic autoimmune rheumatic disease (SAIRD, other than IIM), and non-rheumatic autoimmune disease (NRAID). Basic multimorbidity (BM) was defined as the presence of two or more non-autoimmune comorbidities, while complex multimorbidity (CM) was defined as the presence of three or more such conditions affecting three or more different body systems. Pain VAS scores were divided into mild, moderate, and severe categories for analysis. Differences between groups were evaluated using chi-square test and t-tests. Predictors of worse pain or fatigue were analysed using multivariable negative binomial regression models. Statistical significance was defined as p<0.05.able negative binomial regression models. Statistical significance was defined as p<0.05. Results: A total of 7,843 responses (39.9% healthy, and 13.3% IIM) were analysed. Dermatomyositis (26.5%), females (74%) and Caucasians (45.8%) were most common. The IIM subgroup also included a majority of females (72.8%) and Caucasian individuals (76.8%). Both IIM and SAIRD patients exhibited high pain and fatigue (Figure 1A). While pain levels did not show significant dependence on the IIM subtype, overlap myositis (OM) patients reported higher levels of fatigue (Figure 1B).Overlap myositis patients, those with autoimmune comorbidities, BM or CM, mental health comorbidities, or a longer median disease duration were significantly more likely to report moderate pain levels (Figure 2A). Age and sex did not appear to influence this trend.In contrast, Asian patients exhibited superior outcomes in pain VAS, Fatigue 4A, and PROMIS PF 10 compared to Caucasians. Conversely, Hispanics reported higher pain and fatigue levels (Figure 2B). Additionally, younger patients (<25 years old) demonstrated superior outcomes in pain, fatigue, and overall function compared with their older counterparts (Figure 2B). Conclusion: Patients diagnosed with IIM exhibit heightened levels of both pain and fatigue compared to individuals without these conditions. Factors such as younger age, Asian ethnicity, and shorter disease duration are discernibly linked to lower pain scores among individuals with IIM. Notably, the presence of OM and concurrent comorbidities is associated with unfavourable outcomes concerning both pain and fatigue, thus signifying these subgroups as potential targets for specialized multidisciplinary care interventions. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.Figure 1Demographic details of the study population (1A) and the IIM group (1B); p<0.05 in bold. Figure 2Factors associated with pain perception (2A); Negative binomial regression analysis of factors associated with pain and fatigue (2B)

Background: The treatment of patients with rheumatoid arthritis (RA) has become standardized and targeted in the past decades, using novel disease-modifying anti-rheumatic drugs (DMARDs: biologic (bDMARDs) and targeted synthetic (tsDMARDs)), which resulted in a significant improvement in the disease prognosis. However, treatment patterns might vary around the world according to different patient profiles, medical practices, and socioeconomic considerations. Objectives: To assess worldwide disparities in different DMARDs, with a focus on novel therapies, in patients with RA from the COVAD2 study, taking into account the geographic location and the country's socioeconomic level. Methods: Data on RA patients was extracted from the COVAD2 database, an international multi-centre cross-sectional study based on a patient self-reported online study on patients with autoimmune diseases and controls from 110 countries. Sociodemographic data were collected, and regions of the world were considered in 5 categories: Africa, Asia, Europe, North America grouped with Oceania (due to the low number in the latter), and South America. The Human Development Index (HDI) was used to evaluate the country's economic level. Pain, Patient-Reported Outcome Measurement Information System (PROMIS) scores, Physical function, and Fatigue 4a were recorded. Finally, DMARDs were categorized as corticosteroids, conventional synthetic (cs)DMARDs, Immunosuppressives, Hydroxychloroquine, bDMARDs (TNFi, IL1i, IL6i, IL12-23i, IL17i, IL23i, RTX, Belimumab, CTLA4Igi) and tsDMARDs (JAKi). The distribution of novel therapies (bDMARDs and tsDMARDs) was evaluated by continent and HDI. Factors associated with the novel therapies were identified using Chi-Square, Fisher exact test, and ANOVA as appropriate in a bivariate analysis, and significantly associated variables (p<0.05) were included in a multivariable binary logistic model. Results: At the time of the analysis, the COVAD2 database comprised 10739 participants, including 6390 patients with autoimmune rheumatic diseases and 2007 with RA. In patients with RA, the mean age was 51±14 years, disease duration was 12±10 years, 88% were females, 21% were in disease remission, and 32% had two or more comorbidities. Among all patients with RA, 39% were from Europe, 25% from Asia, 15% from North America and Oceania, 12% from South America and 9% from Africa, with significant differences in age, gender, disease duration, activity, multimorbidity and PROs among the continents. In parallel, 61% were from countries with very high HDI, 24% from high, 13% from moderate and 2% from low HDI. The use of novel therapies was reported in 30% of all patients with RA and was the highest in Europe (44%), followed by North America and Oceania (34%), South America (31%), Asia (12%), and Africa (5%) (p<0.001) (Table 1). Also, it increased with HDI (3% in low, 6% in Medium, 21% in high and 39% in very high HDI countries (p<0.001)). After adjusting on age, disease duration, gender, ethnicity, continent, HDI, pain, disease activity, basic multimorbidity, PROs, the use of novel therapies remained associated with the continent, the country's HDI, and longer disease duration. Conclusion: In patients with RA from the COVAD2 international database, the use of novel DMARDs varied significantly across continents and increased significantly with the country's economic level. These global disparities in RA treatment, beyond patients' and disease's characteristics, should be addressed to ensure equitable access to effective healthcare interventions around the world. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.

Background: Pain plays an essential role in therapeutic decision-making in autoimmune rheumatic diseases (AIRDs). However, it may persist in patients in disease remission, thus impacting quality of life and leading to unnecessary escalation of therapy. Objectives: Using the COVAD2 global database, we aimed to explore the self-reported pain landscape across AIRDs, with a particular focus on patients in disease remission. In addition, we identified the core drivers of pain among patients in remission in regard to socio-demographic characteristics, comorbidities, and patient-reported outcomes (PROs) using validated scales. Methods: The study was an ancillary analysis from the COVAD2 international cross-sectional, multi-centre patient self-reported online study conducted by 157 investigators from 110 countries, collecting PROs in patients with AIDs and healthy controls (HC). Pain was assessed via the 11-point NRS and then transformed into a binary variable using the median as a cutoff. Patients were considered in remission if they self-reported inactive disease or remission and were treated with glucocorticoids daily dose <10 mg/day. The PRO Measurement Information System (PROMIS) score was recorded with global physical health (GPH), mental health (GMH), function and fatigue 4a. We performed latent class analysis (LCA) to reveal hidden profiles of AIRD patients in remission with pain and assessed the association of individual characteristics (namely age, gender, continent and/or ethnicity, disease duration, AIRDs, fatigue, PROMIS GPH, GMH) with membership of latent subclasses using logistic regression. Results: Of 10,739 participants, 6,390 had AIRDs, 572 had non-rheumatic (nr-)AIDs, and 3,777 were HC. Most participants were from Asia, North America and Europe (26% from each), median age 45 years (IQR 35-58), 7,930 (74%) females, median AIRD disease duration 8 years (IQR 3-15), 823 patients (20%) were in disease remission. Among all participants, the mean level of pain was 2.8 (SD 2.6), significantly higher in patients with AIRDs, 3.6 (SD 2.15), compared to nr-AIDs, 2.4 (SD 2.5) and HC, 1.5 (SD 2.5) (p<0.001). In patients with AIRDs in remission, pain was significantly lower compared to those with active disease (2.2 (SD 2.3) vs 3.8 (SD 2.5), p<0.001), but remained higher vs nr-AIDs (1.3 (SD 2.0)) and HC (1.1 (SD 1.7)) (p<0.001). In those patients, a higher level of pain was associated with older age, longer disease duration, female gender, higher HDI, continent (highest in Africa, lowest in Asia), and ethnicity (highest in native Americans and lowest in Asians), type of AIRD (highest in RA, lowest in CTDs), higher fatigue, poorer function, psychiatric comorbidity, basic multimorbidity, PGPH, and PGMH. The LCA identified a 2-class basic model as optimal. The first latent class, with a prevalence of 48.5%, had a high probability of high pain (0.94) and of active disease (0.31). The second one had a prevalence of 51.5% with a low probability of high pain (0.09) and of active disease (0.09). The first class was associated with the type of AIRD (RA, OR 1.2 [95% CI 1.10-1.50] compared to CTDs), the continent (Europe, OR 1.22 [95% CI 1.01-1.48]), higher PGPH (OR 1.19 [95% CI 1.01-1.40] after adjusting on age, gender, PROMOS GPH, PROMIS GMH, Fatigue and disease duration. Conclusion: Data from the COVAD2 study identified significant residual pain in patients with AIRDs in remission status. Determinants of this residual pain were identified and should be considered on a personalized basis in order to avoid unnecessary anti-rheumatic treatment escalation. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.

Background: Since the literature on patient-reported outcome measures (PROMs) in Takayasu arteritis (TAK) is scarce [1], we comprehensively assessed PROMs in a cohort of TAK longitudinally and compared them with healthy individuals. Objectives: •Comparison of PROMs in patients with Takayasu arteritis and healthy controls.•Comparison of PROMs in inactive and active Takayasu arteritis.•To evaluate changes in PROMs on longitudinal follow-up. Methods: Consecutive adult patients with TAK were prospectively evaluated for quality of life (QOL, using EQ-5D-3L questionnaire), physical function [using health assessment questionnaire (HAQ)], physical activity (using International Physical Activity Questionnaire – Short Form (IPAQ-SF)], work productivity [using Work Productivity and Activity Impairment- General Health v2(WPAI)], anxiety [using generalized anxiety disorder assessment 7 questionnaire (GAD-7)], depression [using patient health questionnaire 9 (PHQ-9)], fatigue [using 13-item Functional Assessment of Chronic Illness Therapy Fatigue Scale (FACIT, lower FACIT scores indicate greater fatigue) and Multidimensional Fatigue Inventory (MFI)], and fibromyalgia [using 2010 diagnostic criteria from the American College of Rheumatology (ACR), including the polysymptomatic distress scale (PSD) score]. PROMs were compared between TAK and healthy controls, and between TAK with active or inactive disease as per physician global assessment. PROMs were reassessed in TAK after 6-18 months to assess the stability of observations. Medians with interquartile range (Q1-Q3) were used to represent the data (unpaired data compared using Mann-Whitney U test, paired data using Wilcoxon matched-pairs signed rank test). Categorical data were compared using Chi-square test. p<0.05 were considered significant. Results: Eighty-four patients with TAK [61 females, median age 34(25-44) years, disease duration 66.5 (37.7-125.8) months] were compared with 61 healthy controls [45 females, age 30 (28-40) years]. TAK had worse QOL on EQ-5D visual analog scale and higher scores in domains of mobility, self-care, usual activities, pain/discomfort, and anxiety/depression of the EQ-5D-3L than healthy controls (Figure 1A). Patients with TAK had worse physical function (HAQ), greater activity impairment due to health (using the WPAI), higher anxiety (GAD-7) and depression (PHQ-9) scores, worse fatigue scores (MFI, FACIT) and PSD scores than healthy controls (Figure 2A). Similar proportions of TAK (6/84) or controls (1/61) fulfilled the 2010 ACR diagnostic criteria for fibromyalgia (p=0.239). Active TAK (n=13) had worse scores on the mobility domain of EQ-5D-3L (Figure 1B), higher activity impairment due to health (using WPAI) and worse fatigue (MFI, FACIT) and PSD scores than inactive TAK (n-71) (Figure 2B). Repeat measurements of PROMs in 75 patients with TAK after a median 6.5 (6-9) months revealed no significant differences (Figures 1C and 2C). Conclusion: Patients with TAK have worse patient-reported outcome measures than healthy controls which remain similar on longitudinal assessment. Active TAK have worse fatigue and work impairment than inactive TAK. REFERENCES: [1] Misra DP, et al. Rheumatol Ther 2021;8(3):1073-93. Acknowledgements: NIL. Disclosure of Interests: None declared.Figure 1EQ-5D-3L in TAK. 1A. Comparison between TAK and healthy controls. 1B. Comparison between inactive and active TAK. 1C. Comparison between TAK on longitudinal follow-up. p values for comparison are presented in figures. Figure 2PROMs in TAK. 2A. Comparison between TAK and healthy controls. 2B. Comparison between inactive and active TAK. 2C. Comparison between TAK on longitudinal follow-up. p values for comparison are presented in figures.

Background: The idiopathic inflammatory myopathies (IIMs) are systemic rheumatic autoimmune diseases that pose risk for accelerated atherosclerosis.[1] Although BSR 2022 guidelines advise regular cardiovascular (CV) risk assessment in IIMs, insufficient evidence exists to advise screening threshold and frequency.[2] Objectives: We audited CV risk assessment in patients with IIMs in various regions based on BSR recommendations. We further explored the prevalence and predictors of increased CV risk in IIM patients. Methods: A total of 262 adults (≥25 years) classified as IIMs (ACR EULAR 2017 classification criteria), attending Rheumatology units within the last three years were audited for CV risk assessment. CV assessment was then undertaken for these patients using QRISK3 score and WHO cardiovascular risk charts to identify the unmet need for intervention. QRISK>20% was inferred as high risk. Descriptive statistics were used for data summarization, chi square and ANOVA tests for intergroup comparisons and logistic regression analysis in R (version 4.3.2) was used to estimate the odds ratios. Results: Six cohorts across five regions (India, Hong Kong, Brazil, Italy, and the UK), consisting of 703 patients with IIM were audited. Among these, 262 had data retrievable for the CV risk assessment (Figure 1A). Evidently, CV assessment was not routine at most centres, with risk scores documented in four (1.5%) on the first audit, although lipid levels were often assessed (197, 75.2%), often by GP, more so in some regions with high-risk demographics. The baseline characteristics of the cohorts are summarised inTable 1.Thirty six (13.7%) of the audited IIM patients were classified as high CV risk, of which 26 (72% of high risk, 9.9% of total cohort) were on statin or alternative therapy according to NICE [3] guidelines.Patients in the UK and Italy appeared to have a higher CV risk than those in other regions (Figure 1B, Table 1). An increase in risk was seen with age > 60 years and a reduction in risk for patients with dermatomyositis compared to other IIM categories (when adjusted for age, sex, other subtypes, systolic blood pressure, and body mass index)(Table 1).The Pearson correlation coefficient between the WHO CV risk score and QRISK3 scores in our cohort was 0.358, p<0.01 (Concordance for Low-Low scores 42.2%, high scores 16.2%), suggesting that these capture different aspects of the CV risk spectrum and may warrant further exploration of appropriate screening strategies. Conclusion: The CV risk estimation in this large cohort of IIM patients was suboptimal. Adults with dermatomyositis had a lower CV risk than those with other IIM subsets in this multicentre study. In IIM, one in ten individuals faces CV risk, which is influenced by age and the demographic profile. Effective management requires the establishment of care pathways to address comorbidities and ancillary health issues to ensure holistic care.*This map does not comment on Geo-political bordersTable 1. Baseline cohort characteristics and CV risk assessment REFERENCES: [1] Oreska et al.Cardiovascular Risk in Myositis Patients Compared to the General Population: Preliminary Data From a Single-Center Cross-Sectional Study. Frontiers in Medicine.2022;9:861419. https://doi.org/10.3389/fmed.2022.861419.[2] Alexander et al, British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy, Rheumatology. 2022;61:1760-8, https://doi.org/10.1093/rheumatology/keac115.[3] https://www.nice.org.uk/guidance/cg181. Acknowledgements: NIL. Disclosure of Interests: None declared.

Background: Psychosocial wellbeing significantly contributes to the overall health experiences of individuals with systemic lupus erythematosus (SLE). Mental health is often influenced by the intricate interplay between multifaceted factors, including the impact of disease on quality of life, pain, fatigue, drug-related side effects, and disease-specific manifestations. In view of this complexity, a comprehensive understanding of these factors collectively is required, particularly as some factors may be modified. Objectives: This study aims to identify the key variables contributing to overall mental health in SLE and uncover associations with risks of poor mental health in a global patient population. Methods: Data was extracted from the COVAD database of more than 20,000 survey respondents with autoimmune diseases and healthy controls (HC). Variables including age; ethnicity; disease duration; treatment including glucocorticoids (GC) and immunosuppressive medication (IS); comorbidities; and recent disease flares (particularly relating to the cutaneous and musculoskeletal domains) were evaluated for their association with poor mental health. Mental health was assessed using the Patient-Reported Outcomes Measurement Information System (PROMIS) Global Mental Health, a validated measure of overall mental health accounting for emotional wellbeing, social connections and general life satisfaction. This generates a numerical score (0-20) with a lower score suggestive of worse mental health. We defined poor mental health as a PROMIS Global Mental Health score of ≤11. This was based on data obtained from 3323 HCs included in the study, in which the mean PROMIS Global Mental Health score was 14.80 minus 1 standard deviation (3.31). Differences in characteristics between patients with and without poor mental health were tested for statistical significance using χ2, Mann-Whitney or t-test, as appropriate. A logistic regression model was constructed to evaluate predictors of poor mental health and included age, gender, ethnicity, comorbidity burden, hydroxychloroquine use and flares including rash and/or joint manifestations of SLE. Outputs of the multivariable logistic regression were presented as odds ratios (OR) with 95% confidence intervals (CIs), with graphical representation of marginal probabilities of poor mental health by age band and disease activity. Results: A total of 1292 SLE patients were identified of whom 36.7% (n=474) fulfilled the definition of poor mental health. Table 1 summarises differences in demographics, treatment and comorbidities. Those with poor mental health were older (median 42 years, IQR 32-53 vs 38 years, IQR 31-48, p<0.001), more likely to be female (p=0.029) and reported higher levels of pain (median Pain VAS 5, IQR 3-7 vs 2, IQR 0-4, p<0.001) and fatigue (Fatigue 4a 14, IQR 11-16 vs 8, IQR 7-12, p<0.001). Figure 1A shows the results of the logistic regression model and illustrates that when compared with individuals of black ethnicity (the group with the lowest incidence of poor mental health), white ethnicity exhibited an increased risk of poor mental health (adjusted OR 2.59, CI 1.58-4.25), followed by Hispanic (OR 2.30, CI 1.33-3.96) and Asian ethnicity (OR 2.10, CI 1.29-3.42). Increasing numbers of physical comorbidities also associated with higher risk of poor mental health in the adjusted model (adj OR 1.61, CI 1.31-1.98). Interestingly, active cutaneous disease did not appear to significantly associate with poor mental health (adj OR 1.05, CI 0.53-2.08), whereas those with active joint disease had markedly higher risk of poor mental health (adj OR 3.65, CI 2.38-5.61). Figure 1B shows the probability of poor mental health was greater in those with active lupus disease compared to inactive, and this effect persisted across all age groups. Conclusion: Our findings highlight that more than one third of patients with SLE suffer from poor mental health. We identify important risk factors associating with poor mental health in SLE, such as pain (particularly in the setting of recent flare of joint disease) and fatigue. Effective management of these symptoms may improve mental health outcomes in people with SLE. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: Eman Elfar: None declared, Gagandeep Sukhija: None declared, Katie Bechman: None declared, Sreoshy Saha: None declared, Johannes Knitza: None declared, Carlo Vinicio Caballero: None declared, Praggya Yaadav: None declared, Dey Dzifa: None declared, Ioannis Parodis IP has received research funding and/or honoraria from Amgen, AstraZeneca, Aurinia Pharmaceuticals, Elli Lilly and Company, Gilead Sciences, GlaxoSmithKline, Janssen Pharmaceuticals, Novartis and F. Hoffmann-La Roche AG., Rohit Aggarwal RA has a consultancy relationship with and/or has received research funding from the following companies: Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therepeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio., Vikas Agarwal: None declared, Latika Gupta: None declared, Chris Wincup: None declared.

Background: Consensus guidelines for malignancy screening in idiopathic inflammatory myopathy (IIM) were recently published by an International Myositis and Clinical Studies (IMACS) working group. These guidelines propose different investigation strategies based on ‘high,’ ‘intermediate’ or ‘standard’ malignancy risk groups. Objectives: In this international multicentre study, we assessed the alignment of current malignancy screening practices with the guidelines and examined the economic implications of their implementation. Methods: We conducted a retrospective analysis of malignancy screening practices in IIM patients across six institutions in five countries. Patient demographics, clinical features, and data regarding malignancy screening tests were extracted from the medical records. IMACS risk stratification categories were then determined. Current screening practices and the impact of implementing the new IMACS guidelines were explored using descriptive statistics. Results: Of the 535 IIM patients analysed (mean age 46.5 years (S.D. 15.8); 74.6% female; 6.5% malignancy), 42.4% were classified as high-risk for malignancy, 37.4% were intermediate-risk, and 17.0% were standard-risk. The remainder (3.2%) had inclusion body myositis (IBM) or juvenile-onset DM (JDM) and hence were not considered at risk of IIM-related cancer according to the IMACS guidelines. The most common ‘high-risk’ factors were age > 40 years at diagnosis (64.1%) and dermatomyositis (42.4%). Notably, 55.5% of patients with anti-synthetase syndrome (ASyS) or overlap myositis (OM) were assigned an intermediate- or high-risk classification, predominantly due to the age criterion (age > 40 years in 75.0%, 72/96), although 50.0% of these were only 41-50 years old (36/72). The only cancer detected in ASyS/OM patients was in one individual aged 65 years with significant weight loss.A marked discrepancy was observed between established screening practices and the newly introduced IMACS guidelines. Few high-risk (7.5%), intermediate-risk (6.6%), and standard-risk (27.5%) patients underwent the full panel of recommended IMACS screening tests (Table 2). Among high-risk patients, underutilised tests included mammography (21.4%, 33/154), colonoscopy (23.4%, 53/227), PET/CT (26.4%, 60/227), fecal occult blood test (35.2%, 80/227), gastroscopy (48.0%,109/227), serum CA-125 (48.7%, 75/154), Pap smear (54.5%, 84/154), and serum protein electrophoresis (55.6%, 125/225). Of the 35 patients with malignancy, 94.3% were classified as intermediate- or high-risk however only two underwent full malignancy screening. Regional screening practices varied, possibly reflecting local guidelines. Fifty-nine patients underwent PET/CT without prior CT C/A/P; this practice was most common in Hong Kong. Few high-risk patients underwent annual screening for 3 years as recommended (9.1%, 14/154).There was some evidence of over-screening among the intermediate-risk (6.0%, 12/200), standard-risk (6.6%, 6/91) and IBM/JDM (41.2%, 7/17) cohorts. The most commonly over-screened IIM subtype was ASyS (24.0%, 6/25). Despite this, economic analysis revealed that adherence to the IMACS guidelines would increase screening costs 1.5 to 2.9-fold compared to current practice. The financial impact was greatest in high-risk patients, with a 1.9 to 4.1-fold increase in costs, depending on country. Conclusion: Implementation of the recently proposed malignancy screening will significantly impact clinical practice and healthcare expenditure by increasing the volume and cost of testing. Our findings indicate that malignancy detection may be feasible without strict adherence to these protocols, suggesting the potential for over-screening, pending further exploration in longitudinal cohort studies.Rationalising the guidelines by assigning greater significance to low-risk features and recalibrating the age threshold for otherwise low-risk patients could mitigate these impacts. Targeted physician education regarding the limited value of intensive screening for certain subtypes may further reduce unnecessary testing. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: Jessica Day Research support from CSL PTY, Samuel Katsuyuki Shinjo: None declared, Ho So: None declared, Lekshmi Minikumari Rahulan: None declared, Shereen Oon Project support from CSL PTY LTD, Ian Teh: None declared, Iris Yan Ki Tang: None declared, Edmund Ka Ming Wong: None declared, Pallavi Vij: None declared, Vikas Agarwal: None declared, Latika Gupta: None declared

Background: Idiopathic Inflammatory Myopathies (IIM) are a group of heterogeneous autoimmune diseases that can present with multi-organ involvement. Proper assessment of disease activity level is crucial for tailoring treatment decisions for patients with IIM, especially with the advent of virtual consultations. Outcome measures for such assessments rely on the objective evidence of organ involvement as noted by a physician, but this differ from the patient’s perception. Objectives: We aimed to identify the various factors responsible for the discordance between a patient’s perception of disease activity and a surrogate for their physician’s, to highlight the unmet need for better outcome measures for IIM and pave the way for a solution. Methods: The COVAD-2 e-survey is an international collaborative research study with 15,832 participants (1052 with IIM). Demographic details, IIM subtype, comorbidities, medications, and validated patient reported outcome measures (PROMs) were collected. The IIM diagnoses were self-reported but were made by a rheumatologist, dermatologist, or neurologist. Patient perceptions of disease activity were self-reported as “active” or “inactive.” We inferred a physician’s perception of active disease based on the presence of either one active clinical feature of IIM (muscle weakness, joint swelling, difficulty swallowing, difficulty breathing, elevated muscle enzymes; and rash for the dermatomyositis (DM) subgroup), or the need to start a new or increase the dose of an immunomodulator (including a daily prednisolone equivalent dose >10mg).Patients were divided into “concordant” and “discordant” groups, based on the agreement or disagreement between the patient and physician definitions of “active” or “inactive” disease, respectively. Basic multimorbidity (BM) was defined as the presence of two or more non-autoimmune comorbidities, whereas complex multimorbidity (CM) was defined as the presence of three or more such conditions affecting three or more different body systems. Pain VAS, Fatigue 4A, and PROMIS PF 10 scores were used as functional outcomes. Differences between groups were evaluated using chi-squared or t-tests. Cramer’s V was used to assess the strength of concordance between patient- and physician-reported disease activities. Predictors of discordance were analysed using binomial logistic regression. Statistical significance was defined as p<0.05. Results: Of the 1052 IIM patients, the majority were female (73.3%) and Caucasian (77.2%), with dermatomyositis (DM) being the most common IIM subtype (26.3%). Caucasians comprised the majority of inclusion body myositis (IBM) patients (93.1%). Patients with IBM had a significantly higher mean age, worse PROMIS PF 10 functional status, and were far more likely to have a discordant activity status (Table 1). 773 patients (73.5%) felt that their disease was active, while the physician’s assessment of active disease was met in 450 (42.8%).A total of 578 patients (54.9%) reported a discordant activity assessment status, mainly due to patients perceiving their disease as active as opposed to the physician assessment (43.4%). There was poor overall agreement between patient and physician assessments (Cramer’s V=0.1047, P<0.001).Males, patients with IBM, and the presence of CM were all significantly associated with patients reporting discordance (Table 2A). The discordant patient group reported poorer VAS pain and PROMIS PF 10 outcomes (Table 2B). Conclusion: More than half of the patients with IIM reported a disparity between self-reported disease activity and clinical signs directed/ -immunosuppression denoted inference. Older patients, and those with IBM were statistically more likely to have this discordance. The presence of complex multimorbidity seemed to be a driving factor for discordance, and discordant patients were more likely to have higher levels of pain and worse functional outcomes that likely colour the patient’s experience of their disease.Table 1. Characteristics of the study population (n=1052), p<0.05 in bold.Table 2. Factors affecting discordance in our study (2A); Predictors of discordance (2B). REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.

Background: Patients with autoimmune diseases (AD) are often at risk of severe COVID manifestations and post COVID syndrome (PCS). Pregnant and breastfeeding (PB) women with AD are an even more vulnerable population, though little is known about the prevalence and characteristics of PCS. Objectives: We explored the frequency and characteristics of PCS in the antenatal period in women with AD using the COVAD 2 dataset. Methods: The COVAD 2 is a global initiative with responses from 106 countries gathered as a patient -reported electronic survey translated into 18 languages and vetted by a team of experts. PCS was defined as symptoms persisting for more than 3 months after COVID infection, in accordance with the WHO definition. Sex other than female and incomplete responses were excluded. Frequency of PCS in pregnant/breastfeeding (PB) women with AD was compared to controls without AD. Results: A total of 15832 responses were gathered, of which 7150 were women (46.2%), 2338 (32.7%) reported at least one positive COVID test, and 1417 (60.6%) submitting a complete COVAD survey at least 3 months after a positive test. Females diagnosed with AD were found in 63.3% of the responses. The frequency of PCS in women with AD was higher compared with women without AD (11.8% vs. 7.9%, respectively). Out of the entire cohort analyzed, 45 women (3.2%) had a positive test during pregnancy or during breastfeeding period, 44.4% women being diagnosed with AD (Table 1). The frequency of PCS among PB women was 6.7% (3/45 cases), compared with 10.5% among non-pregnant, non-breastfeeding females. Considering the presence of ADs, the frequency of PCS in PB women with AD was 15%, while no reports of PCS were documented in PB controls. All three women with PCS in PB group had persistent symptomatology for more than 100 days (135, 114, 121 days, respectively) and had a positive COVID test during pregnancy, neither during breastfeeding period. The three cases were diagnosed with myositis, overlap syndrome and connective tissue disease, respectively. Two out of three women had a disease flare during COVID-19 infection. In two cases oxygen supplementation and special advanced treatment for COVID was administered. Conclusion: One in fifteen pregnant women with AD seem to be at risk of PCS, that's higher than in controls, this risk being slightly higher than the background risk identified in individuals with ADs. All cases of PCS in pregnant/breastfeeding women had a positive COVID during pregnancy, therefore it seems plausible that antenatal period may predispose to a more severe condition and longer resolution of the symptomatology. REFERENCES: NIL. Acknowledgements: The authors are grateful to all COVAD Study Group collaborators. Disclosure of Interests: None declared.

Background: Takayasu arteritis (TAK) is a systemic disease characterized by large vessel involvement. Although the vascular characteristics of TAK are well characterized, there is currently a lack of organized research examining the non-vascular manifestations of the disease. Objectives: The objective was to assess extra-vascular manifestations in a large cohort of TAK patients. Methods: Patients diagnosed with TAK from 4 independent cohorts in tertiary referral centres (2 from Italy and 2 from India) were included. Characteristics were retrospectively collected from an electronic dataset. Logistic regression analysis was performed to evaluate the association between vascular and extravascular manifestations. Variables determined statistically significant (p<0.05) at univariate analysis were included in a multivariate analysis. Results: We included 407 patients (163 Italy; 244 India), 79.1% female, with a median follow-up of 80±123 months. Mean age at diagnosis was 32±13, with a diagnostic delay of 20±50 months. Mean baseline Disease Extent Index for TAK (DEI.Tak) and Indian TAK Clinical Activity (ITAS2010) scores were 8.8 ± 5.5 and 10.4 ± 6.6, respectively. Italian patients presented more frequently fatigue (p<0.001), arthralgia (p<0.001), carotidynia (p<0.001), acute coronary syndrome (p=0.017), but less vertigo (p=0.020), stroke (p=0.005), seizure (p=0.032), pulse loss (p=0.002), arterial hypertension (p<0.001), acute kidney injury (p=0.003) and heart failure (p=0.002). Only 0.7% were asymptomatic at diagnosis. At least one TAK extravascular manifestation was observed in 11.3% of patients (23.3% Italy vs 3.3% India, p<0.001), with the most common being erythema nodosum (4.2%), followed by inflammatory bowel diseases (4.1%), uveitis/scleritis (3.7%) arthritis/sacroiliitis (3.2%), oral ulcers (2.1%), peripheral neuropathy (1.4%). 7 (1.7%) patients had 2 concomitant extravascular manifestations, while 4 (1.0%) had 3, and only 1 (0.2%) had 4. Italian patients presented more frequently erythema nodosum (p<0.001) and uveitis/scleritis (p=0.013). Patients with extravascular manifestations were older (p=0.005), with a longer diagnostic delay (p=0.030). No differences were noted in acute phase reactants. On univariate analysis, syncope, retinopathy, and acute coronary syndrome were significantly associated with extravascular involvement (p=0.039, p=0.021, and p=0.018, respectively), while arterial hypertension and heart failure were inversely associated (p<0.001, p=0.023, respectively). On multivariate analysis, retinopathy (p=0.033) and acute coronary syndrome (p=0.046) remained significant. 348 (85.5%) cases received glucocorticoids, 331 (81.3%) conventional immunosuppressants, and 115 (28%) biologics. Those with extra-vascular involvement were treated more frequently with biologics (p<0.001). Vascular procedures were performed in 101 patients (24.8%), with no differences between groups. Conclusion: Extravascular manifestations of TAK are not rare, with a prevalence of 11.3% of patients, and erythema nodosum is the most common extravascular manifestation. Patients with extra-vascular manifestations are older and experience a longer diagnostic delay, significantly associated with the presence of retinopathy and acute coronary syndrome. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.

Background Limited data on the delayed onset effects of COVID-19 vaccination in patients with idiopathic inflammatory myopathies (IIMs) continues to be a significant cause of hesitancy in this vulnerable group. Objectives To analyze the delayed onset adverse events (ADEs) of COVID-19 vaccination in patients with IIMs, other systemic autoimmune and inflammatory disorders (SAIDs), and healthy controls (HCs), using data from the ongoing 2nd COVID-19 Vaccination in Autoimmune Diseases (COVAD) study. Methods A validated patient self-reporting e-survey was circulated by the international COVAD study group (157 collaborators, 106 countries) from Feb to June 2022, collecting respondent demographics, comorbidities, IIM/SAID details, COVID-19 infection history and outcomes, and vaccination details including ADEs. Delayed onset (> 7 day) ADEs (including minor and major ADEs and hospitalizations) were analyzed in patients with IIMs, SAIDs, and HCs, using various regression models. Results 15,165 total respondents completed the survey, of whom 8759 complete responses from vaccinated individuals [median age 46 (35-58) years, 74.4% females, 45.4% Caucasians] were included in the analysis. Of these, 1390 (15.9%) had IIMs, 50.6% other SAIDs, and 33.5% were HCs. 16.3% of IIMs patients reported minor ADEs, and 10.2% experienced major ADEs, and 2.9% required hospitalization. Patients with IIMs had a lower risk of minor ADEs than other SAIDs, though a higher risk of rashes compared to HCs [OR 4.0 (2.2-7.0), p<0.001]. In the IIMs subgroup, patients with active disease, overlap myositis, and ChadOx1 nCOV-19 (Oxford/AstraZeneca) recipients were at a higher risk of ADEs, while those with inclusion body myositis, and BNT162b2 (Pfizer) vaccine recipients were comparatively protected. Patients with IIMs with co-existing SAIDs were at a higher risk of minor [OR 5.2 (3.3-8.2), p<0.001] and major ADEs [OR 2.1 (1.2-3.8), p<0.05] compared to those with IIMs alone. Conclusion Patients with IIMs were at a lower risk of delayed onset COVID-19 vaccine ADEs compared to other SAIDs, though within this patient group, those with active disease, overlap myositis and autoimmune multimorbidity were vulnerable, and warrant close monitoring and long term follow up post COVID-19 vaccination. Reference [1]Furer V, Eviatar T, Zisman D, Peleg H, Paran D, Levartovsky D, et al. Immunogenicity and safety of the BNT162b2 mRNA COVID-19 vaccine in adult patients with autoimmune inflammatory rheumatic diseases and in the general population: a multicentre study. Ann Rheum Dis. 2021 Oct;80(10):1330–8. Acknowledgements COVAD Study Team. Disclosure of Interests Syahrul Sazliyana Shaharir Speakers bureau: Pfizer, Novartis, Bohdana Doskaliuk: None declared, Naveen Ravichandran: None declared, Jessica Day Grant/research support from: CSL Limited, Parikshit Sen: None declared, Samuel Katsuyuki Shinjo: None declared, Mrudula Joshi: None declared, Nelly Ziade Speakers bureau: Pfizer, Roche, Abbvie, Eli Lilly, NewBridge, Sanofi-Aventis, Boehringer Ingelheim, Janssen, and Pierre Fabre, Consultant of: Pfizer, Roche, Abbvie, Eli Lilly, NewBridge, Sanofi-Aventis, Boehringer Ingelheim, Janssen, and Pierre Fabre, Grant/research support from: Pfizer, Roche, Abbvie, Eli Lilly, NewBridge, Sanofi-Aventis, Boehringer Ingelheim, Janssen, and Pierre Fabre, Tsvetelina Velikova Speakers bureau: Pfizer and AstraZeneca, Marcin Milchert: None declared, Sreoshy Saha: None declared, Johannes Knitza: None declared, Ashima Makol: None declared, Kshitij Jagtap: None declared, Vishwesh Agarwal: None declared, Dzifa Dey: None declared, Carlos Enrique Toro Gutierrez: None declared, Carlo Vinicio Caballero: None declared, Vikas Agarwal: None declared, Rohit Aggarwal Consultant of: Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therapeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio., Grant/research support from: Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therapeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio., Latika Gupta: None declared.

Background Notwithstanding the wealth of literature on COVID-19, studies focusing on young people with autoimmune diseases (ADs) are lacking. Despite their younger age, they may have been exposed to immunosuppressants (IS) for longer than older adults, particularly if diagnosed at pediatric age. Objectives To determine early (within 7 days) and late (after 7 days) anti-SARS-CoV-2 vaccine-related adverse events (AEs), post-vaccine disease flares, COVID-19 severity and breakthrough infections (B-INFs) in young people with rheumatic diseases (RMDs) and non-rheumatic (nr)-ADs compared to healthy controls (HC). Methods The EULAR Young PARE definition was used to classify young patients (18-35 years old). Data were captured through the international COVID-19 Vaccination in AD (COVAD) 1 and 2 questionnaires (covering the years 2021 and 2022) and variables differing across groups in univariable analysis were included in multivariable analysis adjusting for baseline factors defined a priori. Results Of 20,685 complete responses, 6010 were from young people (1692 RMD, 400 nrADs, 3918 HC). RMDs and nr-ADs comprised mainly females of Caucasian, Asian and Hispanic ethnicities. Before vaccination, <5% and 7% of nrAD were taking glucocorticoids (GC) or immunosuppressants (IS) other than GC vs 30% GC and >80% IS in RMDs. Over 80% of total responders had received ≥2 vaccine doses. 1) AEs after 1 or 2 doses. Early mild AEs were more frequent in RMDs and nr-ADs compared to HC [RMD vs HC odds ratio (OR) (95% confidence interval (CI)) 2.4 (2.0-3.1); nr-AD vs HC 2.0 (1.4-2.9)]. Injection site pain, headache and fatigue were the most frequent mild AE in all 3 groups, but they were more frequent in nr-AD than in HC and RMD. Fever and chills were also more frequent in nr-AD compared to HC and to RMD. The frequency of late mild AEs after either of the 2 doses was < 10% in all 3 groups with no differences between groups. Severe AEs were < 2% in all groups, with no differences between the first and second dose. 2) AEs after 3 or 4 doses. The frequency of late mild AEs after any dose (1, 2 3 or 4) was < 20% in all 3 groups with no differences between groups. Severe AEs after the third dose were <3%. In the 1417 responders that received a fourth dose, only two (1 RMD and 1 HC) reported AEs (0.1%). 3) Pre-vaccine vs breakthrough infections (B-INFs). The frequency of reported SARS-CoV-2 infections was similar in all groups (RMD=28%, nr-ADs=25%, HC=28%). However, RMD patients reported only one infection more frequently than nrADs and HC, while nrADs reported ≥ 2 infections more frequently than RMD. Pre-vaccine infections were less frequent in RMD vs HC (OR=0.6, 95% CI= 0.4-0.9), but similar in nr-AD vs HC. In contrast, the frequency of B-INFs was higher in RMD vs HC (OR=2.7 95% CI 2.1-3.5), while it was similar in nr-AD vs HC. Regarding the clinical profiles of pre-vaccine infections versus B-INFs, no differences were observed among nrADs whereas in RMD a significantly lower frequency of loss of smell and loss of taste was reported for B-INFs. Less than 5% of total respondents received advanced therapies for SARS-CoV-2 infection. 4) Post-vaccination disease flares: Self-reported disease flares after the second vaccine dose were reported by 10% or RMD and 7% of nrAD patients. Of these, approximately half reported requiring a change in their medications (increased dose/addition of a new IS and/or GC). Conclusion This study provides the first detailed exploration of the SARS-CoV2 spectrum in young people with RMDs and nrADs compared to HC and highlights important similarities and differences between disease groups. In fact, despite being less exposed to GC and IS, nr-AD reported a higher number of SARS-CoV-2 infections, no difference in the clinical picture of pre-vaccine infections vs B-INFs and a more pronounced burden of post-vaccine mild AEs after earlier doses compared to RMD. These findings may highlight that in young people the type of disease rather than IS therapy may be more important to influence the vaccine safety and the features of B-INFs. REFERENCES NIL. Acknowledgements NIL. Disclosure of Interests Alessia Alunno: None declared, Francesco Carubbi: None declared, Ai Lyn Tan: None declared, Parikshit Sen: None declared, Lorenzo Cavagna: None declared, Mrudula Joshi: None declared, Jessica Day: None declared, Kshitij Jagtap: None declared, Sreoshy Saha: None declared, Carlos Enrique Toro Gutierrez: None declared, Carlo Vinicio Caballero: None declared, Oliver Distler Consultant of: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur., Speakers bureau: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur., Grant/research support from: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur., Hector Chinoy: None declared, Rohit Aggarwal Consultant of: Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therapeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio., Grant/research support from: Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therapeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio., Vikas Agarwal: None declared, Latika Gupta: None declared.

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Background A subset of Takayasu arteritis (TAK) begins in the pediatric age group (≤18 years). Differences in prognosis between pediatric-onset and adult-onset TAK are unclear. Objectives To compare the presentation and prognosis between pediatric-onset and adult-onset TAK. Methods The clinical presentation, angiographic features, treatment patterns, disease activity on follow-up, and survival were compared between pediatric-onset and adult-onset TAK. Categorical variables were compared using odds ratios (OR between pediatric-onset and adult-onset TAK, with 95% confidence intervals). Survival for pediatric-onset and adult-onset TAK were presented using Kaplan-Meier plots and compared using log-rank test. Hazards ratios (HR, with 95% confidence intervals) were calculated, both crude and adjusted for prognostic factors. Propensity-score matched analyses adjusted for prognostic factors were conducted to assess adjusted differences in survival between pediatric-onset and adult-onset TAK. A previous meta-analysis of the risk of mortality with pediatric-onset vs adult-onset TAK [1] was updated with the findings from this study. The study was approved by the institutional ethics committee., which waived the requirement for informed consent in view of the retrospective review of chart records. Results Heart failure [odds ratio (OR) for pediatric-onset vs adult-onset TAK 2.76, 95%CI 1.12–6.80] and chest pain (OR 3.03, 95%CI 1.11-8.34) were more common in pediatric-onset TAK, and stroke or transient ischemic attack was more frequent in adult-onset TAK (OR 0.31, 95%CI 0.10-0.93). Hata's angiographic type V (OR 1.95, 95%CI 1.00-3.78) was commoner in pediatric-onset TAK, as opposed to Hata's type I in adult-onset TAK (OR 0.18, 95%CI 0.04-0.79). Disease activity at baseline, 6, 12, and 24 months, the use of glucocorticoids and disease-modifying antirheumatic drugs (DMARDs) were similar between pediatric-onset and adult-onset TAK. The unadjusted hazard ratio for mortality with pediatric-onset versus adult-onset TAK was 6.13 (95%CI 1.51-24.91), and after adjustment for prognostic covariates (gender, delay to diagnosis, baseline disease activity, number of conventional and biologic/targeted synthetic DMARDs used), was 4.97 (95%CI 1.20-20.58) (Table 1). Survival was worse for pediatric-onset than adult-onset TAK on unadjusted analyses (Figure 1) and after propensity score matching for these covariates (54 pairs, log rank p value 0.026). Risk ratio for mortality with pediatric-onset vs adult-onset TAK across studies was 2.27 (95%CI 1.05-4.85) after updating a meta-analysis of four observational studies [1] by including the present study. Conclusion Pediatric-onset TAK associates with greater mortality than adult-onset TAK. Greater vigilance is required while managing pediatric-onset TAK. Reference [1]Misra DP, Rathore U, Kopp CR, Patro P, Agarwal V, Sharma A. Presentation and clinical course of pediatric-onset versus adult-onset Takayasu arteritis-a systematic review and meta-analysis. Clin Rheumatol 2022. Acknowledgements: NIL. Disclosure of Interests None Declared.Figure 1Survival of patients with pediatric-onset and adult-onset Takayasu arteritisTable 1Risk estimates for the association of pediatric-onset TAK (compared with adult-onset TAK) with mortalityCovariates adjusted for using Cox proportional hazards modelHazard ratio (95% confidence intervals)None6.13 (1.51 – 24.91)Gender (male or female)5.93 (1.46 – 24.08)Gender (male or female), Delay to diagnosis5.56 (1.36 – 22.67)Gender (male or female), Delay to diagnosis, disease active at baseline assessment or not5.84 (1.45 – 23.50)Gender (male or female), delay to diagnosis, disease active at baseline assessment or not, number of conventional DMARDs used5.70 (1.42 – 22.920Gender (male or female), delay to diagnosis, disease active at baseline assessment or not, number of conventional DMARDs used, number of biologic DMARDs used4.97 (1.20 – 20.58)n=182 for all modelsDMARDs – Disease-modifying anti-rheumatic drugs