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Abstract Background The clinical manifestations and course of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) exhibits considerable heterogeneity. In this study, we aimed to explore radiographic progression over a defined period, employing the Warrick score as a semi-quantitative measure in early RA-ILD, and to assess the associated risk factors for progression. Methods RA-ILD patients underwent consecutive Warrick scoring based on initial high-resolution computed tomography (HRCT) at diagnosis and the first follow-up. Associations between Warrick scores, pulmonary function tests, and patient characteristics were analyzed. The ROC curve assessed the predictive performance of the Warrick score change rate for ILD progression, while multivariable logistic regression analysis identified risk factors for progression. Results Significant correlations were found between Warrick scores and age at RA-ILD diagnosis, age at ILD diagnosis, and baseline DAS28-ESR. For the severity score, correlations were r = 0.359, r = 0.372, and r = 0.298 (p = 0.001, p < 0.001, p = 0.014, respectively); for the extent score, r = 0.364, r = 0.318, and r = 0.255 (p = 0.001, p = 0.005, p = 0.038, respectively); and for the total score, r = 0.376, r = 0.367, and r = 0.280 (p < 0.001, p = 0.001, p = 0.022, respectively). Annual changes in severity, extent, and total Warrick scores showed sensitivities of 91–97% and specificities of 98% for predicting progression over a 5-year follow-up. Cut-off values were 0.0278 for the severity score (AUC 0.954), 0.0227 for extent score (AUC 0.976), and 0.0694 for total score (AUC 0.946). Warrick severity, extent, and total scores increased significantly during follow-up. Age > 50 years (OR 7.7; p = 0.028) and baseline usual interstitial pneumonia (UIP) pattern (OR 3.1, p = 0.041) were identified as risk factors for progression. Conclusions Advanced age and UIP pattern were significant risk factors for progression. Warrick scoring may may help predict progression in RA-ILD, particularly through changes in severity, extent, and total scores. Due to the retrospective design and small sample size, further prospective studies with larger cohorts are needed to confirm these findings and validate Warrick scoring as a reliable marker for RA-ILD progression.

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Abstract Background To date, there has been limited exploration, particularly on a national scale, of the prevalence patterns of comorbidities and complications associated with rheumatoid arthritis (RA) in Colombia. We aimed to analyse the prevalence patterns of comorbidities and disease-related complications of RA patients enrolled in Colombia’s contributory healthcare regime. Methods We performed a nationwide observational descriptive cross-sectional study using administrative claims data. We used a set of sensitive and specific electronic algorithms (i.e., a set of rules) applied to linked data based on ICD-10 codes and unique medication use codes. We compared all those algorithms with several sources, including governmental agencies and scientific literature, to identify all the known adults treated for RA. Results A total of 123,080 RA cases for 2018 were identified, corresponding to a point prevalence of 0.86 (95% CI 0.86–0.87) per 100. Compared to a non-RA reference population, hypertension (68.2 vs. 20.0%), osteoarthritis (43.6 vs. 6.1%), and osteoporosis (18.6 vs. 1.1%) provided larger standardized mean differences. Lupus (30.04; 95%CI 29.3–30.8), multiple sclerosis (7.18; 95%CI 6.6–7.8), and osteoporosis (5.57; 95%CI 5.5–5.6) provided higher age- and sex-adjusted prevalence ratios. Disease-related complications were found in 62.2% of cases. Conclusions We describe the first comprehensive assessment of the prevalence patterns of disease-related complications and comorbidities that define the RA burden of disease within a multimorbidity profile. Also, our study provides a narrower and more reliable point prevalence estimate for RA in Colombia.

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Abstract Background There is no consensus on the classification of lupus arthritis (LA). In this study, we aimed to investigate patients with LA who were clinically and serologically very similar to those patients with rheumatoid arthritis (RA). Methods The electronic medical files of systemic lupus erythematosus (SLE) patients from a single tertiary rheumatology department between 2017 and 2022 were reviewed. The inclusion criteria were being age ≥ 18 years, having nonerosive peripheral arthritis lasting longer than six months, and having dual seropositive (rheumatoid factor (RF) and anti-citrullinated protein antibody (anti-CCP)) serology. A nonerosive course of arthritis was demonstrated by both conventional radiography and joint ultrasound. Images were assessed by two blinded rheumatologists. Patients with drug-induced lupus and those with other rheumatologic diseases were excluded. Results The cases of 528 patients were reviewed, and eight patients were included in the study. All patients were female, and the median age was 48.5 years. The median SLE and arthritis durations were 12 and seven years, respectively. The most common SLE symptom was photosensitivity (n = 8). Only one patient had life-threatening involvement (LTI), which was a seizure and autoimmune haemolytic anaemia. All patients had arthritis affecting the wrist and hand. Anti-dsDNA was the most common anti-ENA antibody (n = 7), followed by anti-SSA (n = 5). The median RF and anti-CCP titres were 82.5 IU/ml and 81.5 U/ml, respectively. Five patients had high titres of autoantibodies, and only one patient had slight hypocomplementemia. Three patients needed biologic agents, and remission was achieved after treatment with rituximab. Conclusion Despite a long arthritis duration and dual seropositive serology with high titres of RF and anti-CCP, our patients had SLE rather than rhupus syndrome. The low frequency of LTIs, such as lupus nephritis, was a remarkable feature of our patients. Lupus arthritis may be clinically and serologically indistinguishable from RA. Prospective studies are needed to better define LA.

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Abstract Background Fibromyalgia (FM) is a musculoskeletal syndrome characterized by diffuse and chronic pain associated with other symptoms such as fatigue, sleep/cognition disorders, headache, depression and anxiety, resulting from a change in pain processing. Previous research has shown an increase in some interleukins (IL) in patients with FM when compared to controls, however, there is still no uniformity and consensus in the results. There is no study that evaluates IL8 mRNA expression in FM and its association with obesity and other clinical parameters. This study aims to verify the impact of IL8 mRNA expression on the clinical parameters of patients with FM (FMG) in relation to the comparison group (CG). Method This study evaluated patients diagnosed with FM treated at the rheumatology service of the Hospital das Clínicas of the Federal University of Pernambuco (HC-UFPE). The CG group was composed of individuals without chronic pain (companions of the patients and hospital employees). Clinical and demographic data were collected in both groups, and questionnaires for fatigue (FACIT-F), impact of FM (FIQ-R), depression (BDI), and sleep (NRS) were applied to both groups. Peripheral blood was collected for evaluation of IL8 gene expression through real time polymerase chain reaction (qPCR). Results Patients with FM show a lower frequency of IL8 gene expression compared to the CG, but FMG presented mainly up regulated in relation to CG. There was no association of IL8 expression and worse FIQ-R indices, sleep disturbance, BMI or fatigue. However, there was an association between IL8 expression and moderate depression ( p =0.002) and physical activity p =0.039), where patients in FMG who did express IL8 were practicing less physical activity. Conclusion Patients in FMG did not have a higher frequency of IL8 expression compared to CG, however patients with IL8 expression have a greater association with moderate depression.

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Abstract Objective To evaluate the effectiveness of intra-articular injections (IAIs) with triamcinolone hexacetonide (TH) combined with a progressive resistance exercise program (PREP) in improving pain, function, muscle strength, and quality of life in elderly patients with knee osteoarthritis (OA). Methods Fifty-nine elderly individuals with knee OA were randomized into three groups: IAI with TH (IAI-TH) + PREP, IAI with saline solution (IAI-SS) + PREP, and IAI with placebo + PREP. The IAIs were administered once, one week before starting PREP, which was performed twice weekly for 12 weeks. Outcomes assessed at baseline and at 2, 6, and 12 weeks post-IAI included pain (Numerical Pain Scale - NPS), swelling, function (Western Ontario and McMaster Universities Osteoarthritis Index - WOMAC), quality of life (Short Form-36 - SF-36), performance tests (Six-Minute Walk Test - 6MWT, Timed Up and Go Test - TUGT, Short Physical Performance Battery - SPPB), and muscle strength (one-repetition maximum test - 1RM). Due to the COVID-19 pandemic, only 15 participants per group completed the study protocol. Results All groups showed significant intragroup improvements over time in pain, function, muscle strength, and quality of life. However, no statistically significant differences were found between the groups for any of the assessed outcomes. The bodily pain domain of the SF-36 and analgesic consumption were the only measures showing differences over time. Conclusion The combination of IAI-TH and a 12-week PREP (twice weekly) was not superior to IAI-SS or placebo combined with the same PREP in improving pain, function, or quality of life in elderly patients with knee OA. These findings highlight the role of exercise as a key therapeutic strategy, regardless of prior IAI. Future studies with larger sample sizes and long-term follow-ups are needed to better assess the role of intra-articular corticosteroid injections in OA rehabilitation. Clinical trial number ensaiosclinicos.gov.br (RBR-556md5g). Registered 27 October 2022.

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Abstract Background The coexistence of dermatomyositis (DM)/clinically amyopathic DM (CADM) and psoriasis has been infrequently documented in the literature. Consequently, this study aimed to analyze this entity from our tertiary center and review the relevant literature. Methods This retrospective observational cross-sectional study and case series included patients with DM/CADM and psoriasis between 1998 and 2024. A literature review was also conducted. Results Nine of 331 patients with DM (n = 265)/CADM (n = 66) had psoriasis; six were female, and all were of white ethnicity. The median age at DM diagnosis was 38 years (range: 18–78), and at psoriasis diagnosis was 43 years (range: 18–81), with a median interval of four years between diagnoses. The follow-up revealed that six patients were discharged, two died, and one continued follow-up. The primary comorbidities included systemic arterial hypertension (n = 3) and diabetes mellitus (n = 3). Four patients presented with varicella zoster (n = 1) or pulmonary tuberculosis (n = 3). Regarding the literature review, 15 articles reported a total of 17 cases of overlapping DM/CADM and psoriasis. However, variability was observed in the DM/CADM diagnostic criteria. The mean age at DM diagnosis in the literature was 32.3 years (range: 2–59), whereas for psoriasis, it was 31 (7–63) years. Female patients were predominant. Conclusion This investigation identified the coexistence of DMPs, with a median age of 38 years for DM and 43 years for psoriasis. The variability in the diagnostic criteria underscores the necessity for standardized approaches to enhance patient management.

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Abstract Background Due to the rarity of relapsing polychondritis (RP), we described the demographic, clinical, treatment, outcomes, and comorbidities of patients with RP from our tertiary service. Additionally, a literature review was conducted. Methods A total of 47 Brazilian patients with RP between 2000 and 2024 were analyzed. All patient data were collected from pre-parametrized and pre-standardized electronic medical records. A literature review using PubMed with “relapsing polychondritis” as the search term included 25 articles after applying the strict exclusion criteria. Results A total of 47 patients were evaluated. The median age was 40 (34–51) years, with a female-to-male ratio of 1.4:1, and 89.4% were of white ethnicity. The median time from symptom onset to diagnosis was 39 months and the median follow-up duration was 7 years. Ear cartilage biopsy was performed in 12.8% of cases. The clinical manifestations included auricular chondritis, arthralgia, and ocular involvement. Approximately half of the patients had hypertension and dyslipidemia, one-third had diabetes mellitus, and one-fifth had hypothyroidism. Tracheostomy and cochlear implantation were required in 12.8% and 6.4% of the patients, respectively. Disease outcomes showed that 46.8% of patients were in remission, 29.8% had active disease, and 25.5% were controlled with immunosuppressive therapy. Mortality occurred in 6.4% of the cases. In the literature review, 25 studies were analyzed, most of which originated in Asia. Studies have reported the classical manifestations of RP, such as auricular chondritis, arthritis, and ocular involvement. The median age of the patients with RP was similar across studies, averaging 46.4 years, with a predominance of female patients. A comparison with the literature showed consistency in clinical manifestations, particularly auricular chondritis and septum nasal chondritis, although few studies have explored comorbidities, disease evolution, and outcomes. Conclusions The reviewed articles described classical clinical manifestations, but few articles reported data on other manifestations and comorbidities that can occur in RP. Our study provided new insights by mapping symptom evaluations, thereby enhancing the understanding of disease evolution. Understanding and characterizing RP will allow for better assistance in its diagnosis and follow-up.

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Abstract Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood, but its outcomes are still difficult to determine. We aimed to obtain outcome measurements of disease activity, functional capacity, disease damage, and therapeutic response, at one-year follow-up study on a real-life basis. Methods An observational JIA cohort from two referral centers for pediatric rheumatology in Brazil Pediatric Rheumatology Centers was carried out over a period of one year. Clinimetric validated outcome measurements were applied over four visits. Multivariable logistic regression was performed to evaluate baseline variables associated with the following outcomes after one year of follow-up: disease activity, Minimal Disease Activity (MDA), disease flare, remission on medication and remission off medication. Results A total of 127 patients were included in the study. Eighty-three (65.4%) were females, and median time to diagnosis was 6.0 months. JADAS71 and CHAQ improved during follow-up (mean Vbaseline 7.05 ± 9.3 vs. V52 3.52 ± 8.4; 0 ± 0.5 vs. 0.14 ± 0.4, p < 0.001, respectively) as well as JIA-ACR 30, 50, 70 and 90 (Vbaseline 79.8% vs. V52 88.8%; 71.4% vs. 84.1%; 59.7% vs. 77.6%; 49.6% vs. 69.2%, p < 0.05, respectively). Inactive disease was present in 33% of patients at baseline and in 67.9% at V52 (p < 0.001). Remission on medication, remission off medication and MDA were present in 46%, 11%, and 80.6% of patients at V52, respectively. Extra-articular and articular damage were not common at baseline (0.3 ± 0.7 and 1.1 ± 3.4 respectively) and did not decrease significantly during the period of the study. The presence of active joints 46/101 (45.5%) at baseline reduced the chance of the patient achieving remission off medication at the last visit by 84% (OR = 0.16; CI 95% from 0.03 to 0.91, p = 0.039). Conclusion This study showed improvement in clinimetric outcome measurements and therapeutic responses in an inception cohort of JIA patients. The presence of active joints at baseline is related to not achieving remission at last visit. Long-term prospective and multicenter studies are needed to better assess the outcome of JIA and the differences among JIA subtypes.

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Abstract Objectives Fibromyalgia (FM) is a relevant comorbidity in immune-mediated rheumatic diseases (IMRD). Immune mechanisms have been postulated to trigger FM, potentially leading to distinct clinical features compared to FM occurring without IMRDs. This study aims to provide a comprehensive comparison of FM characteristics in patients with and without concomitant IMRD and to evaluate the differences in diagnostic FM criteria between the two groups. Methods A comprehensive dataset of clinical, psychosocial, and sleep variables, along with validated questionnaires, was prospectively collected from 341 patients with chronic musculoskeletal pain syndromes who participated in a rheumatology-led, two-week multimodal inpatient program at the University Hospital of Lausanne between 2018 and 2024. Participants were included if they met either the Fibromyalgia Rapid Screening Tool (FiRST) or the American College of Rheumatology (ACR) 2010 criteria for FM. The disease activity of the underlying IMRD was assessed by two rheumatologists. Data were separately analyzed in two cohorts: one fulfilling the FiRST criteria and the other fulfilling the ACR 2010 criteria. Results Among the participants, 153 patients met the FiRST criteria, of whom 34 also had a history of clinically diagnosed IMRD. Similarly, 149 patients fulfilled the ACR 2010 criteria, with 32 of them also having IMRD. The most common IMRDs were HLA-B27-negative spondyloarthritis (53%), Sjögren's syndrome (16%), HLA-B27-positive spondyloarthritis (9%), psoriatic arthritis (9%), seronegative rheumatoid arthritis (9%), and seropositive rheumatoid arthritis (3%). In 88% of patients with IMRD, the disease was considered inactive. No significant clinical or epidemiological differences were found between FM patients with or without IMRD in either the FiRST or ACR 2010 cohorts, except for a higher prevalence of enthesopathies and childhood pain in patients with concomitant IMRD. FM patients with IMRD had slightly lower FiRST scores, and FiRST-positive IMRD patients showed a better short-term response to the multimodal program, particularly in FABQ-work, BPI-interference, and Pain Catastrophizing Scale scores. However, no significant difference was observed in the Pain Disability Index (PDI) three months post-program. Conclusion HLA-B27-negative spondyloarthritis and Sjögren's syndrome were the most common concomitant IMRDs in this FM cohort. FM patients with and without IMRD exhibited similar clinical and epidemiological features, suggesting a common pathophysiological background. The FiRST criteria, which emphasize central hypersensitization rather than diffuse pain, appear to be a suitable tool for detecting FM in IMRD patients. This may be particularly useful in cases where enthesial pain might interfere with FM diagnosis, and vice versa. Key messages HLA-B27-negative spondyloarthritis and Sjögren's syndrome were the most common IMRDs in this FM cohort. Fibromyalgia patients with concomitant IMRDs exhibit a clinical and epidemiological profile similar to those without IMRDs. Patients with FM and concomitant IMRDs tended to have lower FiRST scores and showed a better short-term response to multimodal treatment. FiRST and ACR 2010 criteria identify similar FM profiles in IMRD patients, supporting FiRST as the preferred screening tool due to its simplicity and focus on central hypersensitization rather than diffuse pain.

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Abstract Background Myocardial fibrosis is an important adverse prognostic marker, however; determinants of myocardial fibrosis in immune-mediated inflammatory diseases (IMIDs) remain poorly defined. We aimed to identify determinants of myocardial fibrosis in patients with IMIDs, as measured by cardiovascular magnetic resonance (CMR) extracellular volume (ECV). Methods Cross-sectional study of 116 patients with IMIDs undergoing clinical CMR at Manchester University NHS Foundation Trust. IMIDs included rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis (SSc), ankylosing spondylitis, psoriatic arthritis and vasculitis. CMR included pre- and post-contrast T1 mapping to measure myocardial ECV, with same day blood sampling. Determinants of ECV were investigated with univariable and multivariable linear regression. Results ECV varied significantly according to IMID diagnosis (ANOVA F statistic 2.80, P = 0.015); ECV was higher in patients with SSc compared to other IMIDs. Major determinants of ECV as a continuous variable were SSc, smoking and body mass index (BMI); regression coefficients 3.33 (95% confidence interval 0.82–5.84), 3.08 (0.73–5.43), and − 0.19 (−0.29 – −0.09) respectively, P < 0.01 (SSc, smoking and lower BMI were associated with increased ECV). Approximately a quarter of the variability in ECV could be explained by these predictors (optimism adjusted R2 0.265). Conclusion SSc is associated with a higher burden of myocardial fibrosis compared to other IMIDs. In patients with IMIDs, independent determinants of myocardial fibrosis were presence of SSc, smoking and BMI. Importantly, participants underwent CMR for clinical indications and may not be representative of IMID populations in the community.

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Abstract Objective To determine the prevalence of metabolic syndrome (MetS) in patients with childhood onset Systemic Lupus Erithematosus (cSLE) and controls from Northeastern Brazil and to verify its association with specific SLE parameters and cardiovascular risk factors. Methods The prevalence of MetS was assessed cross-sectionally in 58 patients with cSLE and 33 age -matched controls. Information was collected by clinical examination and standardized questionnaires, investigating personal and family history of cardiovascular disease and obesity and socioeconomic and demographic characteristics. Results The prevalence of MetS was higher in cSLE patients than in controls according to both ABRAN criteria (8.6% vs. 0%; p = 0.083) and IDF criteria (10,3% vs. 3.0%; p = 0.208), but without statistical significance. Importantly, 91.4% of patients were from a low-income household. Patients with MetS according to ABRAN also had lower ESR levels (p = 0.039), higher total cholesterol (p = 0.013), HDL-c (p = 0.007) and triglycerides (p = 0.001) and a lower albumin level (p = 0.016). Patients with MetS according to IDF had higher SDI scores (p = 0.039) and higher C3 and C4 levels (p < 0.001 and p < 0.001, respectively). The multivariate logistic regression identified higher levels C4 (OR = 32.6; 95% CI = 1.0-544.0; p = 0.015) and increase in the number of leukocytes (OR = 1.9, 95%CI = 1.1–3.2; p = 0.022) as independent risk factors for MetS in patients with cSLE. Conclusion The prevalence of Mets in the patients with cSLE seems to be low in this population. There was association of MetS with higher cumulative damage indices and levels of complement. We did not observe any association with clinical manifestations, autoantibody profile and dose of corticosteroids.

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Abstract Introduction Biosimilars reduce the cost of biologic therapy without compromising safety and effectiveness. In this study we evaluated Brazilian rheumatologists’ knowledge and perceptions of biosimilars. Methods Cross-sectional and descriptive study based on a questionnaire containing 17 items on familiarity, knowledge and perceptions of biosimilars. Results Answers were received from 135 rheumatologists, of whom 97.8% were familiar with biosimilars and 92.5% had at some time prescribed them, but only 47.7% felt comfortable prescribing them to stable patients and 62.2% strongly disagreed with automatic substitution. In addition, 51.9% preferred naive patients when starting treatment with biosimilars. Conclusion Despite the growing acceptance of biosimilars, many physicians remain reluctant. Evidence-based continuing education is essential to clarify these issues.

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Abstract Background Systemic lupus erythematosus (SLE) directly impacts pregnancy outcomes, and few studies have analyzed the related risk factors for maternal and fetal/perinatal complications in Brazil. We described and analyzed the risk factors for maternal and fetal complications in SLE pregnancies at an outpatient clinic in the State of Amazonas, Northern Brazil. Methods Pregnancies that occurred after the SLE diagnosis between 2001 and 2020 were analyzed. Risk factors for adverse outcomes were determined using logistic regression. Results A total of 155 pregnancies from 109 women were included; the mean age was 28.2 (±5.5) years, the median disease duration was 72 [36; 108] months, 56 (36.1%) had active disease prior to pregnancy, 39 (26.5%) had nephritis, 30 (20.3%) had cutaneous manifestations, and the incidence of disease activity during pregnancy was 29.7%; there was a 12.3% (95% CI, [4.2; 20.4]) increase in the proportion of patients with active disease, there were 35 (22.9%) fetal deaths, 26 (16.8%) cases of preeclampsia, 44 (37.3%) preterm births, 16 (16.2%) cases of low birth weight for gestational age, and 18 (18.8%) cases of intrauterine growth restriction. Risk factors for maternal events included immunological alterations (OR=3.02; 95% CI [1.11; 8.21]), renal involvement (OR=3.74; 95% CI [1.25; 11.21]), and disease activity before pregnancy (OR=1.30; 95% CI [1.04; 1.64]); the use of hydroxychloroquine before pregnancy was protective (OR=0.23; 95% CI [0.08; 0.67]). Risk factors for fetal events included the use of acetylsalicylic acid (ASA) during pregnancy (OR=5.22; 95% CI [1.33; 20.54]) and disease activity during pregnancy (OR=1.31; 95% CI [1.14; 1.52]); the use of antimalarials before and during pregnancy was protective (OR=0.14; 95% CI [0.04; 0.44]). According to the post-hoc analysis, the probability of a Type S error in the ASA association was 100%. The retrospective nature and the presence of missing data about laboratory tests are the main limitations of this study. Conclusion Pregnancy management in SLE presents a unique set of challenges that require a comprehensive and multidisciplinary approach. Careful monitoring of disease activity, appropriate medication management, and psychosocial support are essential for optimizing maternal and fetal health outcomes.

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Abstract Background About 5–10% of patients with Sjögren's Disease (SjD) will develop non-Hodgkin's lymphoma, with a 16 to 44 increased risk compared to the general population. The relationship between SjD and other types of cancer is poorly described in the literature. Aim To characterize patients with SjD from the Brazilian Sjögren's Disease Registry (BRAS) who developed hematological and solid neoplasms, analyzing the organ primarily involved, subtypes, occurrence of metastatic disease, timing of diagnosis and correlation with demographic, serologic aspects, and labial salivary gland inflammation. Methods Among consecutive patients included in the Brazilian Sjögren's Disease Registry (BRAS) and fulfilling the 2002 or 2016 classification criteria for SjD, those who developed neoplasia were retrospectively identified and categorized according to the National Cancer Institute (Ministry of Health, Brazil). RESULTS: 1,010 patients were included; of them, 975 were women (96.5%), with an average age of 55.6 ± 13.6 years. Disease duration was 11.9 ± 7.9 years. We found that 114 out of 1,010 patients (11.3%) had cancer, with a higher prevalence in those over 55 years old (p < 0.001). The most prevalent malignancies were skin cancer [24/858 (2.7%)], breast cancer [27/1010 (2.6%)], lymphoma [15/1010 (1.5%)], and thyroid cancer [8/1010 (0.8%)]. Further, 0.9% of patients had more than one type of cancer. Cancer diagnosis followed SjD diagnosis in 66.7% of lymphoma cases, 53.6% of other malignancies, and 75.0% of skin cancer. The presence of cancer was associated with age (OR 1.04). In the case of skin cancer, the duration of SjD was an additional risk factor (OR 1.08). For thyroid cancer, inflammation of the labial minor salivary glands was identified as an associated risk factor (OR 10.1). Conclusions Other types of cancer were more prevalent than lymphoma in our population of SjD patients. Breast neoplasia emerged as the most prevalent after skin cancer, and thyroid cancer was the second most prevalent solid neoplasia. Aging, disease duration and labial salivary gland inflammation were associated risk factors. Prospective cohort studies are essential for assessing other risk factors for cancer development in SjD patients.

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Abstract Background Takayasu arteritis (TAK) is a rare form of systemic vasculitis that primarily affects the aorta and its major branches. Despite several epidemiological studies on TAK, only a few descriptive studies have been conducted in our country, which motivated us to conduct this study. Methods This single-center retrospective cohort study included Brazilian patients with TAK who underwent follow-up at a tertiary outpatient clinic between January 2000 and June 2024. Patient data were extracted from electronic medical records using standardized and parameterized information. Results A total of 203 patients with TAK were included, with a predominance of Caucasians (83.7%) and females (79.8%). The median age at disease diagnosis was 28.0 (interquartile range: 20.0–38.0) years, and the median follow-up period was 122.0 (49.0-177.0) months. During the initial or follow-up period, 65.0% of patients used GC and 80.0% used IM/IB drugs. The three most common Hata classifications were V (46.8%), IV (23.2%), and I (22.7%). There was a high burden of cardiovascular risk factors, including hypertension (72.9%), dyslipidemia (59.1%), and renovascular hypertension (22.7%). When patients were analyzed according to age quartiles, those who were older at the time of analysis predominantly achieved sustained remission without the need for GC or IS therapy. Conclusions This is the largest cohort study to include Brazilian patients with TAK. Even with higher cardiovascular risk factors than in other series, we observed lower rates of ischemic or cardiovascular events. Moreover, disease activity and treatment patterns varied significantly according to patient age at the time of analysis, with older patients being less likely to require ongoing IS therapy.

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Abstract Background Chikungunya fever (CF) is marked by acute, subacute, and chronic phases, with a significant proportion of patients experiencing persistent joint and neuropathic pain. These symptoms may mimic those of rheumatic diseases such as rheumatoid arthritis (RA) and spondyloarthritis (SpA). There is currently no consensus on whether the infection directly causes chronic joint disease or serves as an immunological trigger for the development of rheumatic conditions. Aim This study aims to evaluate the overall proportion of CF patients who progress to chronic arthropathy, and to identify how many of these patients meet the classification criteria for RA and SpA (including ankylosing spondylitis (AS) and psoriatic arthritis (PsA)). Methods A thorough search was conducted in electronic databases, including PubMed, Embase, LILACS, and the Cochrane Library. The primary endpoint was the occurrence of chronic arthropathy, defined as joint signs and symptoms lasting more than six weeks following the acute phase of CF. The secondary endpoint involved the proportion of patients meeting the classification criteria for RA, SpA, AS, and PsA. A random-effects meta-analysis model was utilized to combine studies and determine the pooled frequency of persistent joint symptoms. Subgroup analyses were performed based on the fulfillment of classification criteria for rheumatic diseases. The risk of bias was assessed using the Joanna Briggs Institute critical appraisal tools. The study protocol was registered with PROSPERO (CRD42020211430). Results A total of thirty-eight studies, comprising data from 12.524 individuals with CF published between 2008 and 2022, met the inclusion criteria. Of these, 4.324 (34.5%) patients developed chronic arthropathy; among them, 11.43% (240 in 2099) patients met the criteria for RA, 12.1% (86 in 711) patients for SpA, 3.42% (36 in 1052) patients for AS, and 2.05% (25 in 1220) patients for PsA. Conclusion This study found that approximately one-third of CF patients experience persistent joint pain lasting over six weeks. However, only a minority of these individuals meet classification criteria for rheumatic diseases.

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Abstract Background Chronic pain (CP) in radiographic Axial Spondyloarthritis (r-axSpA) is primarily peripheral nociceptive pain. However, many patients experience persistent pain despite treatment, suggesting other underlying mechanisms, such as central sensitization (CS) and neuropathy. This study assessed the presence of CS and/or neuropathic pain (NP) in r-axSpA patients treated at a University Hospital in the state of Paraiba. Methods This quantitative, descriptive, and cross-sectional observational study used a convenience sample collected over 24 weeks. Instruments included the Central Sensitization Inventory - CSI (positive score ≥ 40); The PainDETECT Questionnaire - PD-Q (positive score ≥ 19); clinical epidemiological questionnaires, and preliminary diagnostic for fibromyalgia (FM) [American College of Rheumatology criteria of 2010/2011, revised in 2016], applied when CS positive. Inclusion criteria: patients diagnosed with r-axSpA; aged ≥ 18. Analyses performed: Descriptive; univariate and multivariate linear regressions; Student's T-test. Results This study included 52 patients (34 men and 18 women), of whom 61.54% had CS. Among these, 65.62% had FM, with an average widespread pain index of 7.87. NP was present in 17.31%, and 15.38% had both CS and NP. The CSI showed a positive correlation with Bath Ankylosing Spondylitis Disease Activity Index - BASDAI ( r = 0.574) and Axial Spondyloarthritis Disease Activity Score - ASDAS ( r = 0.622), while the PD-Q was positively correlated with BASDAI ( r = 0.515) and ASDAS ( r = 0.551). In the univariate analysis, CSI was a predictor ( p < 0.001) of both BASDAI and ASDAS, with 40.63% of patients scoring BASDAI ≥ 4 and 43.75% scoring ASDAS ≥ 2.1. Additionally, 66.67% of patients with PD-Q ≥ 19 had BASDAI ≥ 4 and 55.56% had ASDAS ≥ 2.1. The PD-Q was a predictor of BASDAI and ASDAS ( p < 0.001). Conclusions This study is the first in the Brazilian scientific literature to investigate and demonstrate the occurrence of CS and/or NP in patients with r-axSpA, even when adequately treated. Another finding considered relevant was the demonstration of the presence of CS in 34.38% of patients who did not meet preliminary criteria for the diagnosis of FM, emphasizing the importance of investigating other painful mechanisms in chronic inflammatory joint diseases. These findings represent a first step for future research, in larger samples and in other forms of axial spondyloarthritis.

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Abstract Background This study aimed to develop a new scoring system for predicting recurrent thrombosis in patients with antiphospholipid syndrome (APS). Methods This was a retrospective multicentre cohort study. Patients with APS were followed up from APS diagnosis to either recurrent thrombosis or their last follow-up date, whichever came first. A multivariable logistic regression analysis with a backward elimination was used to develop a new scoring system, incorporating conventional cardiovascular risk factors and antiphospholipid antibody (aPL) profile as covariates. Each covariate remaining in the final step was multiplied by its β coefficient, rounded to the nearest integer, and then summed. A multivariable Cox proportional hazard model, adjusted for medication use, assessed the association between the new scoring system and recurrent thrombosis risk. The accuracy of the new scoring system in predicting recurrent thrombosis was evaluated by estimating the area under the curve (AUC) and comparing it with the adjusted global APS score (aGAPSS). Results A total of 126 patients with APS were included. The median age of the cohort was 40 (interquartile range: 31–55) years and 49.2% were women. Hypertension, type 2 diabetes mellitus (T2DM), and dyslipidaemia were present in 20.6%, 18.3%, and 54.0% of the patients, respectively. 26.2% were current smokers and 23.8% had concomitant systemic lupus erythematosus. Lupus anticoagulant (LA), anti-β2 glycoprotein I (anti-β2GPI) IgG, anti-β2GPI IgM, anti-cardiolipin (aCL) IgG, and aCL IgM were positive in 76.2%, 27.0%, 11.9%, 27.0%, and 6.3% of the patients, respectively. During a median follow-up duration of 41.5 (interquartile range: 21.4–72.0) months, thrombosis recurred in 9 (7.1%) patients. Multivariable logistic regression analysis revealed the following scoring system: modified diabetes global APS score (mdGAPSS) = 1*T2DM + 2*LA + 1*anti-β2GPI. Multivariable Cox proportional hazard model showed that a higher score was significantly associated with an increased risk of recurrent thrombosis (adjusted hazard ratio 2.911, 95% confidence interval 1.090–7.770, p = 0.033). The AUC was higher using the mdGAPSS (AUC = 0.706) than the aGAPSS (AUC = 0.553). Conclusions Compared to the aGAPSS, the mdGAPSS—comprising T2DM, LA, and anti-β2GPI—demonstrated a greater accuracy in predicting recurrent thrombosis in patients with APS.

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Abstract Purpose To investigate the current status of self-perceived burden (SPB) among rheumatoid arthritis (RA) patients and analyze its influencing factors. Methods A cross-sectional study was conducted with 140 RA patients recruited from four tertiary hospitals in Taiyuan city, Shanxi Province. The data was collected through structured questionnaires assessing: General Information Questionnaire, Self-Perceived Burden Scale (SPBS), Activities of Daily Living (ADL) Scale, Social Support Rating Scale(SSRS). Multiple linear regression were employed to identify key predictors of SPB. Results From 112 distributed questionnaires, 104 were returned valid, with a 92.85% response rate. RA patients (40.38%) exhibited a moderate SPB, with a mean score of 29.10 ± 10.27. The primary concern was the emotional impact on caregivers, with the highest scores reflecting worry about caregiver burden (3.33±1.47). Multivariate linear regression analysis identified marital status (β = 0.19, P = 0.028), Activities of Daily Living (β=-0.26, P = 0.006), and family monthly income (β=-0.30, P = 0.002) as significant predictors of SPB in RA patients. Conclusions The findings underscore the moderate but significant SPB in RA patients, with key influences being Activities of Daily Living, marital status, and economic factors. These insights are vital for tailoring supportive care and shaping public health strategies to better serve this patient population. Clinical trial registration number Not applicable.

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Abstract Aim To determine whether glycosaminoglycans (GAG) of cartilage with osteoarthritis promote inflammation through activation of cyclooxygenase (COX)-2 and/or 5-lipoxygenase (ALOX5) pathways. Materials GAG (50 μg/25μL) extracted from the cartilage of 30 patients subjected to total joint arthroplasty secondary to osteoarthritis (OA) or fracture or saline were injected intra-articularly (i.art.) into wild-type or ALOX5−/− SV129 mice; cell counts as well as COX2 and ALOX5 gene expression were assessed in joint washes after 6 h. Results GAG injection promoted acute cell migration, with predominance (> 85%) of mononuclear cells with GAG from women having greater activity than men. GAG injection significantly increased ALOX5 but not COX2 gene expression in cells of the joint washes, as compared to control. Injection of GAG from patients that sustained a fracture did not alter cell influx in ALOX5-/- mice but cell counts were significantly lower following injection of GAG from OA patients (P = 0.0016). Conclusion GAG promote inflammatory cell migration into joints through triggering of the 5-lipoxygenase pathway. Sex issues may be linked to an increased inflammatory potential of GAG from female OA patients.

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Abstract Introduction Regulatory B cells (Bregs) play a pivotal role in the immune modulation of rheumatoid arthritis (RA). The levels of CD19+CD24hiCD38hi Bregs in peripheral blood and their association with clinical indicators in RA patients have not been extensively studied. The aim of this study was to investigate the alterations in peripheral blood CD19+CD24hiCD38hi Breg levels in RA patients and their association with clinical indicators. Additionally, we explored the variations in Breg levels before and after treatment. Methods Overall, 90 patients with newly diagnosed RA and 32 healthy controls were prospectively enrolled. Symptoms and laboratory findings were collected. Flow cytometry and three-color fluorescence antibody labeling were employed to detect CD19+CD24hiCD38hi Breg levels in peripheral blood mononuclear cells (PBMCs). The proportion of CD19+CD24hiCD38hi Bregs among mature B lymphocytes was analyzed, and correlations with clinical indicators were examined. A follow-up study of 26 RA patients was conducted to compare changes in Breg levels before and after six months of treatment. Results The levels of CD19+CD24hiCD38hi Bregs in RA patients were 89.63% of those in healthy controls. No significant differences in Breg levels were observed among patients with different disease activities. There was a positive correlation between CD19+CD24hiCD38hi Breg levels and morning stiffness duration (r = 0.227, p = 0.027), and negative correlations with age and anti-CCP antibody titers (r=-0.234, p = 0.022; r=-0.218, p = 0.036). No significant correlations were found between Breg proportions and other clinical indicators. The follow-up study showed a decrease in disease activity scores and a significant increase in CD19+CD24hiCD38hi Breg levels after six months of treatment. Conclusions These findings suggest a potential association between Breg levels and the pathogenesis and inflammatory activity of RA. CD19+CD24hiCD38hi Bregs may serve as potential biomarkers for evaluating disease activity and treatment efficacy. Further research is warranted to explore the underlying biological mechanisms and clinical significance of Bregs in RA.

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Abstract Background There is a lack of information regarding body composition (BC) measurements by Dual X-ray emission absorptiometry (DXA) in pediatric age group, mostly normative data to allow comparison among different populations and establish cut-off values. Aging itself, several chronic diseases and use of concomitant medications are associated with changes in BC, which can lead to cardiovascular events, metabolic complications, increased risk of functional loss, falls and death. Our purpose of the study is to establish BC normative data by DXA in healthy Brazilian children and adolescents. Methods Healthy, eutrophic/overweight children and adolescents aged between 5 and 19 years were included. Medical conditions that could interfere with bone mass or BC were excluded. Participants underwent a clinical interview including details about food intake, sun exposure and physical activity. In addition, anthropometric data, pubertal stage analysis, and lumbar spine and whole body DXA measurements using Hologic QDR 4500 were performed in all participants. Individuals were divided into 4 groups: female and male with two age intervals each (between 5 and 9 years and between 10 and 19 years). BC parameters were bone mineral density (BMD), bone mineral content (BMC), total lean mass (TLM), appendicular lean mass (ALM), appendicular lean mass index (ALMI), fat mass (FM), fat mass index (FMI) and android to gynoid fat ratio (A/G) to obtain normative data according to age and gender. Results A total of 349 healthy volunteers were eligible and enrolled for the study. Females had significantly higher values of fat mass values than males. All BC measurements were significantly higher in pubescent than prepubescent girls and boys. Conclusion Our data provided BC normative data for healthy male and female Brazilian children and adolescents. Clinical trial number Not applicable.

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Abstract Background Chronic fatigue severely compromises the quality of life in patients with granulomatosis with polyangiitis (GPA). Traditional diagnostic methods are often time-consuming, relying on clinical expertise and detailed questionnaires. This study aimed to develop a machine learning model capable of predicting chronic fatigue in GPA patients based on clinical data, with a particular focus on improving diagnostic capacity in regions with limited access to specialists. Methods This cross-sectional study collected data on fatigue (measured by the Modified Fatigue Impact Scale, MFIS), functional ability (Health Assessment Questionnaire, HAQ), disease activity (Birmingham Vasculitis Activity Score, BVAS), comorbidities, medication use, physical activity (International Physical Activity Questionnaire - Short Form, IPAQ-SF), and demographic characteristics. Four machine learning algorithms—logistic regression, decision tree, random forest, and extreme gradient boosting—were assessed using a 70/30 train-test split. Model performance was evaluated using area under the curve (AUC), accuracy, F1 score, recall, and precision. Statistical comparisons were performed using Welch's t-test and the Wilcoxon-Mann–Whitney U test for continuous variables, while the chi-square test or Fisher's exact test was applied to categorical variables, with significance set at P < 0.05. All analyses were conducted using R version 4.4.1 for Windows. Results Forty-five patients were assessed: 62.2% were female, with a median BMI of 27.72 kg/m2 (23.2–30.1), a median age of 55.5 years, and a median disease duration of 12.0 years (6.0–17.0). Fatigue was reported by 20 patients (MFIS score ≥ 38), and seven patients (15.5%) had active disease according to the BVAS, which was similar between the fatigued and no fatigued groups (P > 0.05). The fatigued group had more acute-phase reactants and prednisone use (P < 0.05). The tree-based models achieved an AUC of approximately 0.80, outperforming the other models. Conclusion Tree-based models demonstrated superior predictive performance in identifying chronic fatigue. The Random Forest model, in particular, highlighted higher disability in activities of daily living (HAQ), older age, and longer disease duration as key predictors. Although the models performed well, additional data and incorporation of clinically relevant variables may further enhance predictive accuracy. Patients with GPA who experienced fatigue showed higher glucocorticoid use and elevated acute-phase reactants, despite similar levels of disease activity, suggesting mechanisms beyond inflammation. Machine learning shows strong potential as a clinical tool for fatigue identification, especially in settings with limited access to specialist care. Trial registration Universal Trial Number (UTN): U1111-1271-6003; Brazilian Clinical Trials Registry (ReBEC): RBR-9n4z2hh. Registration date: January 18, 2022, and Plataforma Brasil (CAAE # 41762820.1.0000.0068).

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Abstract Background TNF-inhibitors like adalimumab (ADA) have been vital for managing rheumatoid arthritis (RA) for over two decades. With the emergence of biosimilars, real-life evidence is crucial to confirm their sustained efficacy after switching from originator drugs. This study aims to provide comprehensive evaluation of the clinical outcomes of transitioning from adalimumab reference product (ADA-RP) to the biosimilar adalimumab–AACF (ADA-AACF) in patients with RA over a 12-month period in a real-world analysis. Methods This observational study included RA patients who had been on ADA-RP for at least three months before switching to ADA-AACF. Disease activity parameters, including DAS28-CRP, CDAI, SDAI, and CRP-levels, were assessed at baseline (T0) and compared at 6 (T6) and 12 months (T12) following the switch. Results Sixteen patients were included, with a mean duration of ADA-RP use of 69.9 months. DAS28-CRP remained stable when comparing T0 to T6 [2.36 (1.92–2.84) vs. 1.83 (1.72–3.16), p = 0.988] and T12 [2.36 (1.92–2.84) vs. 2.54 (2.09– 2.86), p = 0.874], with similar results for SDAI and CDAI (p > 0.05). The frequency of low disease activity was consistent during the follow-up: 81.3% at T0; 68.8% at T6 (p = 0.617) and 81.3% at T12 (p = 0.480) for DAS28-CRP < 3.2. The same pattern was observed for CDAI ≤ 10 and SDAI ≤ 11 (p > 0.05). CRP-levels and prednisone doses did not show significant variation (p > 0.05) across time points. The biosimilar retention rate was 87.5%, with two patients discontinuing biosimilar before the 12-month mark. Conclusion This real-world study demonstrates the feasibility and sustained efficacy of transitioning from ADA-RP to ADA-AACF in RA patients, reinforcing its role in long-term disease management. These findings in an ethnically diverse population contribute to the growing global evidence on biosimilar adoption, supporting their integration into routine clinical practice and expanding treatment accessibility.

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Abstract Background Endoplasmic reticulum stress (ERS) and the unfolded protein response (UPR) are adaptive mechanisms for conditions of high protein demand, marked by an accumulation of misfolded proteins in the endoplasmic reticulum (ER). Rheumatic autoimmune diseases (RAD) are known to be associated with chronic inflammation and an ERS state. However, the activation of UPR signaling pathways is not completely understood in Sjögren’s disease (SD). This study evaluated the expression of ERS-related genes in glandular tissue of patients with primary SD (pSD), associated SD (aSD) with other autoimmune diseases, and non-Sjögren sicca syndrome (NSS). Methods In a cross-sectional study, minor salivary gland biopsies were obtained from 44 patients with suspected SD and 13 healthy controls (HC). Patients were classified as pSD, aSD, or NSS based on clinical, serological, and histological assessment. Histopathological analysis and mRNA expression analysis of genes associated with ERS and UPR (PERK, XBP1, ATF-6, ATF-4, CANX, CALR, CHOP, and BIP) were performed on the samples. Differences between groups (pSD, aSD, NSS, and HC) were assessed. The influence of chloroquine (CQ) on the ER was also investigated. Results Twenty-eight SD patients showed increased expression of PERK(p = 0.0117) and XBP1 (p = 0.0346), and reduced expression of ATF-6 (p = 0.0003) and CHOP (p = 0.0003), compared to the HC group. Increased expression of BIP (p < 0.0001), PERK (p = 0.0003), CALR (p < 0.0001), and CANX (p = 0.0111) was also observed in the SD group compared to the NSS group (n = 16). Patients receiving CQ(n = 16) showed a significant increase in ATF-6 (p = 0.0317) compared to patients not taking the medication (n = 29). Conclusions Altogether, the results suggest a greater activation of the ERS and UPR genes in patients with SD, especially in the pSD group. Antimalarial drugs, like CQ, used to treat RAD, may affect the ER function in exocrine glands.

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Abstract Objectives To compare the impact of COVID-19 on the clinical status and psychological distress of patients with immune-mediated rheumatic disease (IMRD) caused by SARS-CoV-2 infection with that of noninfected IMRD controls during a 6-month follow-up period. Methods The ReumaCoV Brazil is a longitudinal study designed to follow IMRD patients for 6 months after COVID-19 (patients) compared with IMRD patients without COVID-19 (controls). Clinical data, disease activity measurements and current treatments regarding IMRD and COVID-19 outcomes were evaluated in all patients. Disease activity was assessed through validated tools at inclusion and at 3 and 6 months post-COVID-19. Fatigue, using FACIT-F (Functional Assessment of Chronic Illness Therapy) and psychological distress, using DASS 21 (Depression, Anxiety and Stress Scale - 21 Items), used to evaluated psychological distress, were evaluated at 6 months after COVID-19 in both groups. The significance level was set as p < 0.05, with a 95% confidence interval. Results A total of 601 patients were evaluated—321 patients (IMRD COVID-19 + patients) and 280 controls (IMRD COVID-19- patients)—who were predominantly female with similar median ages. Disease activity assessment over a 6-month follow-up showed no significant difference between cases and controls. Although the mean activity scores did not differ significantly, some patients reported worsened disease activity post-COVID-19, particularly in rheumatoid arthritis (RA) (32.2%) and systemic lupus erythematosus (SLE) patients (23.3%). Post-COVID-19 worsening in RA patients correlated with medical global assessment (MGA) and CDAI scores, with a moderate to large effect size. Diabetes mellitus showed a positive association (OR = 7.15), while TNF inhibitors had a protective effect (OR = 0.51). Fatigue, depression, anxiety, and stress were significantly greater in patients than in controls. Worse disease activity post-COVID-19 correlated with worse FACIT-F and DASS-21 scores in RA patients. No significant associations were found between COVID-19 outcomes and post-COVID-19 disease activity, FACIT-F or DASS-21. Conclusions Post-COVID-19 IMRD patients exhibited significant fatigue, depression, anxiety, and stress, which can be mistaken for disease activity, despite having similar disease activity scores. The variability in reports on IMRD flares and the potential triggering of SARS-CoV-2 for autoimmune manifestations underscore the need for detailed clinical assessment and a comprehensive approach to managing them.

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Abstract Background Anti-PM/Scl autoantibody has been associated with an overlap between polymyositis (PM) and systemic sclerosis (SSc). However, due to limited studies, the relevance of this autoantibody in patients with idiopathic inflammatory myopathies (IIMs) without SSc was analyzed. Methods This single-center retrospective cohort study was conducted between 2004 and 2024. A total of 93 adult patients with IIMs (66 with dermatomyositis and 27 with PM - EULAR/ACR 2017) without SSc were included: 16 anti-PM/Scl(+) and 77 anti-PM/Scl(-). Patients with other types of IIMs, cancer-associated myositis, or overlap myositis, including SSc, as well as those with other myositis-specific and/or myositis-associated autoantibodies were excluded. Results The median age, sex distribution, and median follow-up duration were comparable between the anti-PM/ Scl(+) and anti-PM/Scl(-) groups. There were no differences in clinical and laboratory characteristics, except for a higher frequency of lung involvement, joint involvement, "mechanics’ hand," "hiker's feet," and Raynaud's phenomenon, in contrast to a lower frequency of facial rash and "V"-neck sign in patients with anti-PM/Scl(+) than in those with anti-PM/Scl(-) (all P < 0.05). Furthermore, patients with anti-PM/Scl(+) exhibited a higher frequency of disease relapse (68.8% vs. 33.8%), disease activity (50.0% vs. 24.7%), and immunosuppressant use (methotrexate or azathioprine) at the last medical evaluation (all P < 0.05). Severe infection and death rates were comparable between the groups. Conclusions Anti-PM/Scl positivity was observed in 17.2% of the sample analyzed in the present study. Patients with this autoantibody present clinical manifestations resembling anti-synthetase syndrome, with increased disease relapse and activity rates.

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Abstract Background Numerous inflammatory complications related to COVID are described, including the Multisystem inflammatory Syndrome in Children (MIS-C) and Hyperinflammation. There is a scarcity of studies comparing these two groups. Methods Retrospective longitudinal outcome-conditioned study. Demographic, clinical, and laboratory variables are analyzed. Patients with history of COVID contact or infection with at least 24 h of fever, two or more systems involved and up to 21 years were included. Patients with no laboratory signal of inflammation or with other diagnoses for the condition were excluded. Demographic and laboratory data are presented as medians with interquartile ranges. Dichotomous variables and prevalences are reported as percentages. A ROC curve analysis was conducted to assess the discriminatory ability of these tests in relation to the MIS-C and hyperinflammation groups. Results We present fifty-four patients, thirty-one with MIS-C and twenty-three with hyperinflammation. The most frequent symptom in the MIS-C group was altered mental status in 61% vs. 46% ( p = 0.014) and conjunctival hyperemia in 29% vs. 4% ( p = 0.032). The most frequent laboratory findings were hypoalbuminemia in 68% vs. 26% ( p = 0.002), increased serum troponin in 42% vs. 26% ( p = 0.034), increased d-dimers in 94% vs. 76% ( p = 0.015), as well as increased BNP in 55% vs. 17% ( p = 0.02). On the other hand, the hyperinflammation group more frequently presented respiratory dysfunction in 57% vs. 13% (p = < 0.001) and serum ferritin equal or greater than 500 ng/mL in 94% vs. 77% ( p = 0.046). Conclusions This is an original study comparing clinical and laboratory findings between MIS-C and hyperinflammation due to COVID. Altered mental status is more frequently associated with MIS-C while respiratory symptoms are associated with hyperinflammation. In addition, regarding laboratory tests, there is hypoalbuminemia, increase in serum troponin, BNP, and D-dimers specially in the MIS-C group and hyperferritinemia in the hyperinflammation group. Further studies are needed to assess the cutoff point of biological markers such as BNP, troponin, and d-dimers for diagnosis and/or prognosis in the pediatric population with MIS-C.

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Abstract Background Rheumatoid arthritis is a chronic autoimmune disorder characterized by joint destruction and inflammation. Persistent pain is a common symptom, often impacting quality of life, physical function, and mental health. Despite its prevalence, limited guidance exists for managing chronic rheumatoid arthritis-related pain. This systematic review and meta-analysis aimed to evaluate the effectiveness of various analgesic therapies for managing rheumatoid arthritis-related pain symptoms. Methods A comprehensive search of PubMed, EMBASE, Cochrane, and ClinicalTrials.gov was conducted from database inception through November 7, 2024. Eligible studies included randomized controlled trials and prospective cohorts reporting pain outcomes in adult rheumatoid arthritis patients. Pain score data was stratified by scale (5-point, 10-point, 100-point) and analyzed using a random-effects model. Results Twenty-six studies covering 52 treatment regimens were included. Etodolac demonstrated significant pain reduction on a 5-point scale (200 mg: 3.24; 95% CI: 2.86 to 3.63; p < 0.001; 300 mg: 3.35; 95% CI: 2.96 to 3.74; p = 0.00). Piroxicam also showed benefit (20 mg: 3.35; 95% CI: 2.91 to 3.78; p < 0.001). On the 10-point scale, only extracorporeal shock wave therapy significantly reduced pain (3.36; 95% CI: 2.25 to 4.48; p < 0.001). Celecoxib (Celbesta) (200 mg BID) was most effective in the 100-point subgroup (1.73; 95% CI: 1.32 to 2.15; p < 0.001). Heterogeneity was high (I2 = 91.1%, 94.1%, 60.2%; all p < 0.05), and publication bias was present. Conclusion Several therapies demonstrated substantial pain relief, but heterogeneity, publication bias, and varying study quality limit generalizability. High-quality, long-term trials with standardized pain assessments are needed to guide personalized rheumatoid arthritis pain management. Clinical trial number PROSPERO CRD42024593971, 4 October 2024.

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Abstract Background In rheumatoid arthritis (RA), the persistence of pain despite adequate inflammatory control may indicate the involvement of nociplastic and/or neuropathic pain (NP). Objective To investigate the presence of central sensitization (CS) and/or NP in patients with RA. Methods This descriptive, cross-sectional study used convenience sampling and included adult patients (≥ 18 years) with RA diagnosed according to the 2010 classification criteria of the American College of Rheumatology – ACR. The following instruments were applied: Central Sensitization Inventory (CSI), PainDETECT Questionnaire (PD-Q), and the 2010/2011 ACR criteria for fibromyalgia (FM), revised in 2016 (the latter applied only to patients with CSI ≥ 40). Descriptive and predictive analyses were conducted with a significance level of 5%. Results A total of 113 patients were included (mean age 54.3 years; 93.81% female; mean disease duration 12.11 years). CS was identified in 59.29% of patients; among them, 47.76% met FM criteria, while 52.24% did not. Among patients with CS, 53.73% reported severe pain (Numeric Rating Scale, NRS), and 44.78% had high disease activity (Clinical Disease Activity Index, CDAI). CSI was a significant predictor of both NRS and CDAI in univariate (p < 0.001) and multivariate analyses (p = 0.037 and p = 0.009, respectively). According to PD-Q, 17.70% of patients had probable NP, and PD-Q scores were predictive of CDAI, NRS, and CSI (p < 0.001). Among patients with CS, 26.87% also showed overlapping NP. Conclusions A substantial proportion of RA patients presented with CS-related pain, even in the absence of FM, suggesting a mixed ("top-down and bottom-up") nociplastic pain profile. Both CS and NP were associated with higher pain levels and increased disease activity. This is the first Brazilian study to characterize the coexistence of CS-related pain (with or without FM) and NP in RA, offering relevant insights for the clinical management of chronic pain in this population. These findings reinforce that routine screening and assessment of CS and NP should be an integral and continuous component of RA management, preceding therapeutic adjustments and extending throughout follow-up.

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Abstract Background We aimed to conduct a meta-analysis of randomized controlled trials (RCTs) to examine the efficacy of biologic DMARDs in improving the clinical response of patients with polyarticular JIA. Methods The literature and relevant reviews were searched for published clinical studies comparing the efficacy of standard therapy without biologic DMARDs to that of biologic DMARDs in patients with polyarticular JIA. The focus was on the minimal clinical effectiveness criteria of the pediatric American College of Rheumatology (pedACR30–100), time to disease flare, remission clinical, inactive disease, and pedACR30/flare. This meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Results In total, 9 randomized controlled trials were included in the study. Compared with standard therapy, treatment with biologics significantly improved PedACR70 (relative risk [RR] 1.68; 95% confidence interval [CI] 1.43-– 1.96; p < 0.00001). Similarly, PedACR30 (RR 1.37; CI 1.19–1.58; p < 0.0001), PedACR50 (RR 1.49; CI 1.25–1.77; p < 0.00001), PedACR90 (RR 1.67; CI 1.34–2.09; p < 0.00001) and PedACR100 (RR 1.88; CI 1.05–3.35; p = 0.03) were also significantly improved with biologic treatment. However, patients on standard therapy had worse flare outcomes, as determined by the PedACR30/Flare (RR 0.56; CI 95% 0.45–0.70; p < 0.00001). Additionally, the time to disease flare was significantly shorter with standard therapy (hazard ratio [HR] 0.38; 95% CI 0.27–0.54; p < 0.00001). Conclusion Compared with standard therapy, biologic DMARDs lead to significant and long-term improvements in signs and symptoms in polyarticular JIA patients. Trial registration This meta-analysis was registered in PROSPERO under the registration number CRD42023494938 on December 18, 2023.

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Abstract Objectives To investigate whether sagittal imbalance, defined by sagittal vertical axis (SVA) using EOS® imaging, is associated with spinal mobility, function, and quality of life in patients with axial spondyloarthritis (axSpA). Methods Patients with axSpA were cross-sectionally assessed for sagittal imbalance (SVA ≥ 50 mm). Spinal mobility (BASMI), function (BASFI) and quality of life (ASQoL) were compared between patients with and without sagittal imbalance. Multivariable analyses examined the associations between SVA and the above-mentioned disease outcomes, adjusted for confounders. Mediation analysis explored whether sagittal alignment mediated the relationship between spinal mobility and structural damage. Results Among 117 patients (mean age 51 (SD 11) years, 68% males), 44 (38%) had sagittal imbalance. SVA was only independently and significantly associated with BASMI but not with BASFI or ASQoL. SVA minimally mediated the relationship between mSASSS and BASMI. The optimal BASMI cutoff to identify patients with sagittal imbalance was 5.2 with 80% of correct classification. Conclusions Sagittal imbalance is associated with impaired spinal mobility but not with impaired function or quality of life. These findings may reflect compensatory mechanisms for sagittal balance in long-term axSpA patients. Impaired spinal mobility can be used to identify patients with sagittal imbalance who may benefit from physiotherapy or rehabilitation. Clinical trial number Not applicable. Key points Sagittal imbalance is not associated with function or quality of life in ax-SpA. Only 4.7% of the effect of mSASSS on BASMI was mediated by sagittal vertical axis. The optimal BASMI cutoff to identify patients with sagittal imbalance was 5.2.

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Abstract Background Although using Rituximab (RTX) and intravenous immunoglobulin (IVIg) alone or sequentially is a well-established treatment for several autoimmune diseases, the combination of these two forms of therapy is still rare, and its use is poorly studied. Aim To perform a systematic review on the use of RTX associated with IVIG in autoimmune conditions. Methods PubMed/MEDLINE, EMBASE, and Scielo databases were screened for articles on RTX plus IVIg in autoimmune diseases until May 2024. Results The review encompassed 21 studies evaluating RTX and IVIg for autoimmune diseases. Ten studies focused on pemphigus, involving 85 patients with diverse subtypes (47 pemphigus vulgaris, 27 pemphigoids, and 11 other variants). Most were case reports or series, with one retrospective study including controls. Positive outcomes were reported across all but one case of paraneoplastic pemphigus. Infections, such as P. jirovecii pneumonia, were noted in three studies, highlighting a potential risk. The other 11 studies involved 24 patients with conditions like polyneuropathies, lupus with CNS involvement, and neuromyelitis optica. While most reported favorable outcomes, one trial on IVIg-dependent polyneuropathies found RTX ineffective in reducing IVIg needs. Adverse events included pneumonia, venous thrombosis with pulmonary embolism, and infusion reactions, demonstrating the need for careful monitoring. Conclusion RTX plus IVIg seems to be an alternative option for the treatment of refractory autoimmune diseases. However, more studies with a larger number of participants and in different autoimmune diseases are desired.

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Abstract Introduction Systemic sclerosis (SSc) often leads to decreased muscle strength and mass, impairing physical performance and causing disability. Interventions with resistance exercise (RE) is an effective non-pharmacological approach to mitigate these issues. This systematic review aims to evaluate the effects of interventions with RE on muscle strength, muscle mass, physical performance, physical disability, and quality of life (QOL) in SSc patients, as well as to assess its adherence and safety. Methods A systematic review and meta-analysis were conducted based on a PICOS framework: Patient = Systemic Sclerosis; Intervention = Resistance exercise; Study design = Randomized clinical trials. Searches were performed across MEDLINE (PubMed), PMC, Web of Science, Cochrane Library, LILACS, and EMBASE up to January 2025. Results Ten randomized clinical trials, including 422 participants (∼85% female), were eligible for analysis. Participants’ ages ranged from 42 to 64 years, with body mass indices between 22.5 and 28.0 kg/m2. The intervention period was standardized to 12 weeks. Interventions with RE significantly improved muscle strength (SMD = 2.76 kg; 95% CI, 1.32 to 4.20; p = 0.0002) and functional disability (SMD = −0.47; 95% CI, −0.93 to −0.00; p = 0.05) compared to controls. Interventions with RE also showed superiority in the physical component of QOL (SMD = 0.42; 95% CI, 0.04 to 0.81; p = 0.03). Although enhanced physical performance was observed, statistical pooling was not possible due to limited data. Interventions with RE had a low incidence of adverse events, but data on disease progression and adherence were insufficient. Conclusion Interventions with RE benefits muscle strength, physical function, and QOL in SSc patients, though optimal protocols and adherence strategies need further investigation. More robust studies are required to refine training methods and enhance clinical trial designs.

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Abstract Introduction Transition clinics are conceived as programs dedicated to the active, multidimensional development of a process that addresses the medical, psychosocial, educational, and vocational needs of pediatric patients suffering from a chronic disease that will persist into adulthood. Their understanding is justified in physiological, psychological, and sociocultural terms on the basis of the differential morbidity and mortality associated with a chronic disease that begins in childhood and prevails into adulthood. Materials and methods Here, we reflect on the history, structure, and impact of transition clinics in pediatrics, with an emphasis on pediatric rheumatologic diseases. Additionally, we propose comprehensive reflection as an alternative for the patient, their family, and the medical team, outlining guidelines for development, implementation, and evaluation. Results The transition of care should commence in early adolescence, considering each patient’s cognitive ability as a condition for the initiation of an educational process involving introspection into the disease. Interdisciplinarity is defined as a team that addresses the clinical, physical, emotional, and social dimensions of each patient and their interaction with the environment within the framework of individualized care and family support. Despite this, the lack of evidence supporting standardized guidelines for the implementation and overall effectiveness evaluation of these interventions was highlighted. Conclusions The transition process is considered successful when the patient is adherent and has a positive and informed perception of their health–disease journey. We urge the generation of evidence documenting the comprehensiveness of processes inherent to transition clinics as the foundation of necessity.

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Abstract The article "Brazilian Recommendations for the Management of Tuberculosis Infection in Immune-Mediated Inflammatory Diseases" by Viviane de Souza et al. presents important discussions on the subject; however, the recommendation for annual repetition of the TST/IGRA test for three years and after medication changes raises considerable concerns. This approach may lead to overdiagnosis and overtreatment of latent tuberculosis infection (LTBI). Frequent false-positive results in retests can result in unnecessary antibiotic use, contributing to bacterial resistance, a problem of global significance. The recommendation, considered to have a moderate level of evidence, is subject to criticism. Arguments used to support retesting, such as high conversion rates of tests after one year, reports of tuberculosis cases despite negative screenings being attributed to false negatives, and reliance on other sources with lower levels of evidence, do not constitute sufficient evidence to confirm tuberculosis infection or justify the recommendation. On the other hand, there is evidence that has not been considered in the discussion against the recommendation for retesting, indicating that this practice may increase the risk of diagnosing false-positive infections, leading to overtreatment without clinically proven benefits. Potentially harmful interventions should not be implemented without solid evidence to support them. In this letter to the editor, we briefly discuss this recommendation and the arguments against its implementation, highlighting its associated risks.

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Abstract Background Neurological and psychiatric manifestations occur in patients with primary Sjögren’s disease (SjD) with a wide-ranging clinical presentation, affecting quality of life, social participation, and prognosis. Despite this, neither central nor peripheral neurological symptoms are systematically evaluated in the context of autoimmunity or identified as manifestations of SjD. The EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) covers only part of them in the neurological domain. Methods We performed a systematic review of the diagnosis and prevalence of central, peripheral, and autonomic nervous system manifestations in primary SjD, following the recommendations proposed by the Cochrane Collaboration Handbook. Observational studies were included when their main issue was the diagnosis and the prevalence of the manifestations individually. We employed a generalized linear mixed model (GLMM) method with a random-effects model, and the results were computed using logit transformation, implemented through the ‘meta’ and ‘metafor’ packages in the R software (version 3.6.1). To present these recommendations, agreement among experts was investigated using the Delphi method in in-person meetings. Results We propose ten recommendations regarding the investigation and management of neurological involvement in SjD that had 100% agreement among participants. Conclusion These recommendations add to the literature on the clinical care of patients with SjD.

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Abstract Background The risk of tuberculosis infection (TBI) and its progression to tuberculosis disease (TBD) among persons with immune-mediated inflammatory diseases (IMID) results from a complex interplay of patient and disease characteristics, immunosuppression level, and the epidemiological context. Brazilian recommendations are unclear about TBI screening and its preventive treatment (TPT) in persons with IMID. Objective To provide a comprehensive and evidence-based guideline for managing TBI in persons with IMID in Brazil. Methods This task force was constituded by 42 specialists with interest in IMID and TBD. A core leadership team (CLT) drafted fourteen clinical questions on the risk of tuberculosis and indications of TPT among persons with IMID who started, or are about to start immunosuppressive drugs. The CLT supervised the systematic reviews and formulated the recommendations. The experts voted using the Delphi Method. Results Nine recommendations were established. More than 80% of panelists voted “agree” and “strongly agree” with all statements. In brief, all persons with IMID starting or about to start immunosuppressive treatment should undergo tuberculin skin testing (TST) or interferon-gamma release assays (IGRAs), a chest imaging test and investigation of contact with active pulmonary or laryngeal TBD. TPT is mandatory for those with any positive result after excluding TBD. Exceptions include individuals with a history of TBD or a past positive TBI infection test. IGRA is preferred only in persons BCG-vaccinated in the past 2 years. Those with inconclusive IGRA results can have the test repeated once, and TPT should be offered if it remains indeterminate. TST or IGRA should be repeated yearly, for three years, when the previous test was negative, when starting or changing to a different class of immunosuppressive drug. Overall, the included studies had a low quality of evidence and high risk of bias. Conclusions These guidelines are meant to improve the management of TBI in IMID. Health professionals must consider the epidemiological risk, host features, the social scenario, the characteristics of the disease, the access to health resources, and the development of an individualized plan for every patient.