OBJECTIVE: A sub-population of patients with polymyalgia rheumatica (PMR) require early glucocorticoid (GC) escalation to achieve remission or have a relapsing disease course. This study aimed to investigate predictors o...OBJECTIVE: A sub-population of patients with polymyalgia rheumatica (PMR) require early glucocorticoid (GC) escalation to achieve remission or have a relapsing disease course. This study aimed to investigate predictors of early GC escalation, risk of relapse, and treatment after year 1 with GCs. METHODS: Clinical and biochemical parameters from a prospective cohort of newly diagnosed patients with PMR were analysed. Diagnosis was confirmed after 1 year of follow-up. Outcomes were need for GC escalation to achieve remission between week 2 and week 4, relapse between week 10- and 1-year, and ongoing prednisolone treatment at 1-year follow-up. Prediction models with cut-off estimation were generated using ROC-curves. RESULTS: Sixty patients with a confirmed PMR diagnosis after 1-year of follow-up were included. CRP showed the most consistent predictive signal across all outcomes. CRP cut-off values for early prednisolone escalation were 41.5mg/L at baseline and 22.5mg/L at week 1 with sensitivities/specificities of 78%/73% and 88%/94%, respectively. CRP cut-off levels for predicting relapse were 49.5mg/L at baseline and 7.5mg/L at week 1 with sensitivities/specificities of 65%/86% and 100%/68%, respectively. Ongoing prednisolone treatment at 1-year demonstrated similar results as relapse. Refractory disease within 4 weeks significantly increased the risk of future relapse (OR: 3.7, 95%CI (1.01-10.58)), and ongoing prednisolone treatment at 1-year follow-up (OR: 4.84, 95%CI (1.51-15.5)) CONCLUSION: Monitoring of treatment response within the first one week after diagnosis may help identify patients requiring early glucocorticoid escalation and those at risk of a more severe disease trajectory and may be part of individualized management of PMR.
BACKGROUND: Surgical treatment remains necessary for selected patients with tophaceous gout, particularly when mechanical limitation, nerve compression, ulceration, infection, deformity, or failure of conservative treatm...BACKGROUND: Surgical treatment remains necessary for selected patients with tophaceous gout, particularly when mechanical limitation, nerve compression, ulceration, infection, deformity, or failure of conservative treatment is present. However, postoperative outcomes after surgery for tophaceous gout have not been comprehensively quantified. This systematic review and meta-analysis evaluated postoperative complication profiles and recurrence burden after surgical treatment for tophaceous gout. METHODS: A systematic search of PubMed, Embase, Web of Science, and the Cochrane Library was conducted from database inception to March 25, 2026. Original studies reporting postoperative outcomes after surgical treatment for tophaceous gout were included. Pooled event rates with 95% confidence intervals (CIs) were calculated using a random-effects single-arm meta-analytic approach. Primary outcomes were postoperative infection, delayed wound healing, and recurrence. Secondary outcomes were reoperation, amputation, and overall complications. Functional outcomes were summarized descriptively. RESULTS: 18 retrospective studies were included. The pooled postoperative infection rate was 11.3% (95% CI 8.0%-15.7%), delayed wound healing 9.9% (95% CI 5.1%-18.2%), and recurrence 8.5% (95% CI 4.7%-14.9%). Secondary pooled rates were 9.7% (95% CI 5.5%-16.7%) for reoperation, 3.7% (95% CI 2.0%-6.8%) for amputation, and 24.0% (95% CI 14.0%-38.1%) for overall complications. Significant subgroup differences were identified only for infection according to anatomic site and intervention type. Sensitivity analyses showed that pooled estimates were robust. The certainty of evidence was very low for all outcomes. CONCLUSIONS: Surgical treatment for tophaceous gout is associated with measurable postoperative risks, particularly infection and overall complications. These findings support careful perioperative counseling, structured postoperative surveillance, integrated long-term urate-lowering management, and more standardized reporting of perioperative risk factors and postoperative outcomes in future surgical studies of tophaceous gout.
OBJECTIVE: Anti-RNA-polymerase-III (ARA) and anti-topoisomerase-I (ATA) autoantibodies are associated with diffuse cutaneous SSc (dcSSc). ARA is associated with rapidly progressive skin thickening, scleroderma renal cris...OBJECTIVE: Anti-RNA-polymerase-III (ARA) and anti-topoisomerase-I (ATA) autoantibodies are associated with diffuse cutaneous SSc (dcSSc). ARA is associated with rapidly progressive skin thickening, scleroderma renal crisis (SRC), and gastric antral vascular ectasia, while ATA is associated with severe interstitial lung disease (ILD). However, these associations were derived from heterogenous SSc cohorts. As SSc is now often diagnosed earlier, we aimed to ascertain whether these relationships hold true in early dcSSc and to examine autoantibody associations with organ involvement in early dcSSc. METHODS: The Prospective Registry of Early Systemic Sclerosis (PRESS) includes adults with dcSSc ≤2 years from the first non-Raynaud's phenomenon symptom who met 2013 ACR/EULAR classification criteria for SSc. Participants were enrolled at 12 U.S. academic centers and evaluated every 6 months. Those with dual-positivity were excluded. Differences across autoantibody groups were assessed using appropriate parametric and nonparametric tests. RESULTS: Of 303 enrolled, 233 participants were included (108 ARA+, 68 ATA+, 57 double-negative). Mean age was 51 years; 68% were female. Mean disease duration was 1.2 years. ATA+ participants had lower baseline FVC% predicted (76.0%vs 86.0% ARA+, p = 0.0011) and higher prevalence of ILD (69.6%vs 44.3% ARA+, p = 0.0029). ARA+ participants had higher baseline mRSS (25.0 vs 17.9 ATA+, p < 0.0001) and higher prevalence of SRC (14.4%vs 0.0% ATA+, p = 0.0024). Only the double-negative group had improvement in FVC% predicted over time (+1.39% per year, p = 0.03). CONCLUSIONS: Autoantibody-specific differences in organ involvement were evident early. The double-negative group uniquely showed improvement in lung function over time.
Cantera Estefanía R, Gálvez Sánchez R, Gorostidi Álvarez I
… +16 more, Cruz Barquín H, García Ruiz R, Herrero López M, Flores García JA, Oviedo Madrid M, Abando Casuso M, García Ascacíbar A, Gabrie Rodríguez LG, Yáñez San Segundo L, López Hoyos M, Domínguez-García JJ, González Ponte ML, González-Mesones Galán B, Irure Ventura J, Hernández Hernández JL, Martínez Taboada VM
INTRODUCTION: The 2023 ACR/EULAR antiphospholipid syndrome (APS) criteria reduced IgM weighting and introduced a category for isolated lupus anticoagulant (LA), creating uncertainty about primary thromboprophylaxis in aP...INTRODUCTION: The 2023 ACR/EULAR antiphospholipid syndrome (APS) criteria reduced IgM weighting and introduced a category for isolated lupus anticoagulant (LA), creating uncertainty about primary thromboprophylaxis in aPL carriers with <3 laboratory points. This study evaluates efficacy and safety of antiplatelet therapy and predictive value of the new ACR/EULAR thrombotic risk profiles. MATERIAL AND METHODS: All asymptomatic carriers of single or confirmed LA, anticardiolipin (aCL), or anti-β2-glycoprotein I antibodies (anti-β2GPI), between January 2018 and March 2024 were included (n = 985). Primary outcomes were thrombotic-free survival (TFS), grade ≥3 bleeding-free survival (BFS), and bleeding symptoms. Logistic and Cox regression models were applied, and Altman-Andersen method estimated the number needed to treat (NNT) and harm (NNH). RESULTS: During a median follow-up of 2.84 [IQR 1,12-4,74] years, venous (VTE) and arterial (ATE) thromboses occurred in 4.6% and 4.1% of patients, respectively. Among the entire cohort, aspirin (ASA) improved TFS (HR 0.51; p = 0.001) without affecting BFS (HR 0.96; p = 0.81). P2Y12 inhibitors were associated with higher ATE (OR 13.17; p < 0.001) and minor bleeding (OR 4.875; p = 0.007). High-risk venous profiles predicted VTE (OR 2.42; p = 0.005). Among 458 carriers with <3 laboratory points, ASA remained protective (HR 0.53; p = 0.047) without increasing minor or major bleeding. P2Y12 inhibitors showed no benefit in TFS and were linked to decreased BFS (HR 4.211; p = 0.008). CONCLUSIONS: Low-dose aspirin may lower thrombotic risk without increasing bleeding in asymptomatic aPL carriers, even in those with <3 laboratory points. P2Y12 inhibitors demonstrated reduced efficacy and an increased risk of bleeding, warranting confirmation in future studies.
Fibromyalgia (FM) exhibits substantial clinical heterogeneity, yet reproducible symptom phenotypes with relevance for treatment personalization remain poorly defined. This study examined whether distinct FM phenotypes em...Fibromyalgia (FM) exhibits substantial clinical heterogeneity, yet reproducible symptom phenotypes with relevance for treatment personalization remain poorly defined. This study examined whether distinct FM phenotypes emerge consistently across medicated and unmedicated individuals. We analyzed 558 adults meeting the 2016 ACR criteria recruited from two academic centers. Participants completed standardized assessments of pain, central sensitization, depressive symptoms, sleep disturbance, and physical and emotional function. Multidomain variables were standardized and evaluated using hierarchical clustering, k-means algorithms, principal component analysis, and heatmap visualization. Phenotype stability was compared between those not receiving centrally acting medications at the time of assessment and those receiving centrally acting agents. A consistent three-cluster solution emerged. Cluster 1 represented a low-severity phenotype with relatively preserved function. Cluster 2 demonstrated prominent affective-sleep dysregulation with moderate pain and sensitization. Cluster 3 reflected a high-severity/global impairment phenotype across all symptom domains. Importantly, these phenotypes appeared in both medicated and unmedicated subgroups, indicating that similar symptom phenotypes were observed in participants receiving and not receiving centrally acting medications at the time of assessment. Fibromyalgia is characterized by three reproducible, clinically interpretable phenotypes that demonstrate substantial similarity across medication-status These findings support a precision-based framework for FM classification and provide a foundation for phenotype-guided therapeutic and mechanistic research.
BACKGROUND: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease frequently associated with hand involvement, leading to significant functional impairment. Imaging techniques provide unique opportuniti...BACKGROUND: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease frequently associated with hand involvement, leading to significant functional impairment. Imaging techniques provide unique opportunities to visualize and quantify structural and functional abnormalities of the hand, supporting diagnosis, monitoring, and treatment evaluation. This systematic review summarizes the imaging techniques used in SSc. METHODS: A systematic search of PubMed and Embase was conducted. Eligible studies included original research articles in English that applied or evaluated imaging techniques of the hands in SSc, published after 2000. Ultrasound and nailfold capillaroscopy were excluded, given their established use. Screening was performed independently by two authors. Findings were synthesized by clinical manifestations, study quality was assessed using the QUADAS-2 tool. RESULTS: Sixty-one studies met the inclusion criteria. In total, 25 distinct imaging techniques were identified, enabling assessment of various hand structures, including vascular involvement, inflammation, fibrosis, calcifications, erosions, and bone marrow edema. Vascular imaging was most extensively studied, particularly in the context of Raynaud's phenomenon and digital ischemia, with multiple techniques demonstrating impaired perfusion and altered thermoregulatory responses. MRI consistently detected subclinical inflammatory and erosive changes of joints and soft tissues,. CT-based techniques provided detailed assessment of calcinosis cutis, while optical and photoacoustic methods showed promise for quantifying skin fibrosis. CONCLUSION: Imaging techniques provide valuable, complementary insights into hand involvement in SSc, often revealing subclinical disease. Despite promising results, limited standardization and longitudinal validation currently restrict clinical implementation. Future studies should focus on harmonizing protocols and validating against clinically meaningful outcomes.
OBJECTIVE: Our study aims to evaluate the performance of an unsupervised machine learning approach, linear combinations of principal components (LPC); a rule-based approach, phenotype risk score (PheRS); and M34 Internat...OBJECTIVE: Our study aims to evaluate the performance of an unsupervised machine learning approach, linear combinations of principal components (LPC); a rule-based approach, phenotype risk score (PheRS); and M34 International Classification of Disease (ICD) code counts for identifying systemic sclerosis (SSc) and predicting mortality from electronic health record (EHR). METHODS: We conducted a single-center retrospective study. We developed SSc-specific PheRS and LPC score using 17 clinical features. We then evaluated these methods for identifying rheumatologist-diagnosed SSc and predict mortality. RESULTS: Of 653 patients with at least one M34 code, 547 (84%) had rheumatologist-diagnosed SSc. When compared with randomly sampled matched controls, LPC outperformed PheRS in identifying rheumatologist-diagnosed SSc (AUROC 0.90 vs 0.87). However, in a three-method comparison among individuals with at least one M34 code, LPC was only marginally superior to PheRS and M34 code counts in distinguishing rheumatologist-diagnosed SSc from M34 non-SSc controls (AUROC 0.72 for LPC, 0.70 for PheRS and 0.70 for ICD code counts). Using at least two M34 codes had a sensitivity of 87.8% and positive predictive value of 87.0% in identifying SSc. Despite the limited performance gains for SSc identification, both LPC and PheRS had substantially higher performance in predicting mortality compared to M34 code counts in patients with SSc (AUROC 0.74 vs 0.72 vs 0.63). CONCLUSION: M34 code counts achieved reasonable performance for SSc identification and comparable to those of LPC and PheRS. However, both LPC and PheRS demonstrated better performance for predicting mortality compared with M34 code counts in patients with SSc.
OBJECTIVES: First metatarsophalangeal (MTP) joint osteoarthritis (OA) is a common and disabling foot condition, but the relationship between localised joint changes with pain severity is poorly understood. This study aim...OBJECTIVES: First metatarsophalangeal (MTP) joint osteoarthritis (OA) is a common and disabling foot condition, but the relationship between localised joint changes with pain severity is poorly understood. This study aimed to investigate the association between joint changes on magnetic resonance imaging (MRI) with pain severity in individuals with first MTP joint OA. METHODS: This cross-sectional study used baseline data from 80 participants aged 36 to 74 years (mean [SD] = 57.5 [8.8]) with first MTP joint OA enrolled in two randomised trials. MRI atlas features assessed included osteophytes, bone marrow lesions, bone cysts, effusion-synovitis, cartilage loss, and joint space narrowing. Pain severity was measured using a visual analogue scale. Regression analyses included linear, multivariable models, with exploratory stepwise forward-selection analyses to identify MRI features associated with pain severity. RESULTS: Osteophytes, joint space narrowing, bone marrow lesions in the proximal phalanx and sesamoids, cysts, and effusion-synovitis were not associated with pain severity in unadjusted or adjusted analyses. In contrast, first metatarsal head bone marrow lesions (involving >66% of the first metatarsal head) were significantly associated with pain severity in adjusted (p = 0.025) and subsequent stepwise analyses (p = 0.039). Cartilage loss was additionally associated with pain severity in the stepwise analysis (p = 0.047). CONCLUSION: MRI-detected bone marrow lesions in the first metatarsal head and cartilage loss are associated with pain severity in first MTP joint OA. These findings provide insights into local mechanisms contributing to pain severity in the first MTP joint OA and may support the development of targeted interventions. Further research is needed to clarify the temporal relationship between MRI-detected pathology and symptoms.
OBJECTIVE: To characterize the clinical features, treatment response, and mortality of patients with systemic sclerosis (SSc) associated fibrosing myopathy. METHODS: In this retrospective study, we identified patients en...OBJECTIVE: To characterize the clinical features, treatment response, and mortality of patients with systemic sclerosis (SSc) associated fibrosing myopathy. METHODS: In this retrospective study, we identified patients enrolled in the Johns Hopkins Scleroderma Center Research Registry with histopathological evidence of fibrosing myopathy. Data were collected regarding SSc features, treatment exposures, and outcomes. Muscle involvement was characterized using muscle enzyme levels, muscle magnetic resonance imaging (MRI) findings, electromyography (EMG) data, and histopathologic features. RESULTS: 16 patients were included in the analysis. 14/16 patients (87.5%) received treatment specifically for muscle disease. Response based on muscle strength testing was evaluated at 6-12 months after treatment initiation. 9/16 patients (56.3%) had significant improvement, 4/16 (25%) showed no significant improvement, and 3/16 (18.8%) had inadequate follow-up. The most commonly used treatments were intravenous immunoglobulin, mycophenolate, and rituximab. After a mean follow-up time of 5.5 ± 4.7 years, 8/16 patients (50%) were deceased, with a cardiopulmonary cause of death in 4/8 (50%) of these patients. The baseline median CK value was 97 U/L (interquartile range 46-300 U/L). Myopathic changes were identified in 14/15 patients (93.3%) who had an EMG performed, while muscle edema was identified in 11/12 patients (91.7%) who underwent an MRI. A notable histopathological finding was myofiber atrophy in the perifascicular region in 9/16 patients (56.3%). CONCLUSION: SSc-associated fibrosing myopathy is a distinct entity associated with a high mortality rate in our cohort of patients. Early recognition and initiation of treatment is important due to the potential for improvement in muscle strength.
González-Juanatey C, Llorca J, Castañeda S
… +5 more, Ferraz-Amaro I, García-Gómez C, Román-Ivorra JA, González-Gay MÁ, onbehalf of the CARMA Project Collaborative Group
OBJECTIVE: To analyze the prevalence of cardiovascular-kidney-metabolic (CKM) syndrome and the relationship with cardiovascular (CV) events in rheumatoid arthritis (RA) patients from the CARdiovascular in RheuMAtology (C...OBJECTIVE: To analyze the prevalence of cardiovascular-kidney-metabolic (CKM) syndrome and the relationship with cardiovascular (CV) events in rheumatoid arthritis (RA) patients from the CARdiovascular in RheuMAtology (CARMA) project followed prospectively for 10 years. METHODS: Between July 2010 and January 2012, patients with RA and a comparison cohort of individuals without inflammatory diseases (controls) were recruited from 67 hospitals across Spain. CKM stage at recruitment was classified according to the American Heart Association consensus definition. Cox proportional hazards models adjusted for age and sex were used to evaluate the association between CKM stage and the composite outcome of CV events or all-cause mortality at 10-year follow-up. RESULTS: The study comprised 704 patients with RA and 604 controls followed prospectively for 10 years. The distribution of CKM stages differed significantly between two groups. Controls were more frequently classified in CKM stages 0 and 1, whereas RA patients predominated in advanced stages, particularly CKM stages 3 and 4 (p < 0.001). The incidence of CV events or all-cause mortality increased markedly with advancing CKM stage in both RA patients and controls. CKM stage 4 was associated with a significantly increased risk of composite outcome compared with CKM stage 2 in RA patients (Hazard ratio [HR] 3.90; 95% confidence interval [CI] 2.29-6.64) and controls (HR 5.22; 95% CI 2.39-11.40). RA patients classified as CKM stage 2 experienced a significantly higher risk of adverse composite outcome compared with controls at the same CKM stage (HR 2.40; 95% CI 1.42-4.05, p < 00.1). CONCLUSIONS: CKM syndrome has a high prevalence and is a determinant of CV outcomes in patients with RA.
BACKGROUND: Soluble urokinase plasminogen activator receptor (suPAR) is linked to organ damage in systemic lupus erythematosus (SLE). We investigated whether serum suPAR associates with histological activity and chronici...BACKGROUND: Soluble urokinase plasminogen activator receptor (suPAR) is linked to organ damage in systemic lupus erythematosus (SLE). We investigated whether serum suPAR associates with histological activity and chronicity in diagnostic and 12-month repeat kidney biopsies in lupus nephritis (LN). METHODS: suPAR levels were determined using ELISA in serum samples from 23 LN patients (19 incident) before and 12 months after initiation of immunosuppressive therapy, and from 15 age- and sex-matched healthy controls. Correlations with NIH activity index (AI) and chronicity index (CI) scores were assessed using Spearman's rank correlation coefficient. RESULTS: suPAR levels were higher at active LN than in controls (5.37±0.93 ng/mL versus 2.64±0.97 ng/mL; p<0.001), decreased by month 12 (3.77±0.94 ng/mL; p=0.005), but remained elevated compared with controls (p<0.001). Baseline suPAR did not correlate with AI at baseline (rho=0.202; p=0.368) or month 12 (rho=0.332; p=0.165), whereas month-12 suPAR correlated with month-12 AI (rho=0.595; p=0.007). Baseline suPAR correlated with CI at baseline (rho=0.541; p=0.008) and month 12 (rho=0.554; p=0.017). Month-12 suPAR showed a trend towards a correlation with month-12 CI (rho=0.440; p=0.066). Baseline suPAR correlated with baseline interstitial fibrosis (rho=0.422; p=0.044) and predicted month-12 glomerular sclerosis (rho=0.580; p=0.011) and tubular atrophy (rho=0.477; p=0.045). Month-12 suPAR correlated with month-12 glomerular sclerosis (rho=0.572; p=0.013). CONCLUSIONS: Serum suPAR levels were elevated in active LN and, despite a decline, after 12 months of treatment compared with healthy individuals. suPAR levels correlated with intrarenal chronicity and predicted post-treatment glomerular sclerosis and tubular atrophy, suggesting suPAR as a potential biomarker of kidney damage in LN.
Berthelsen DB, Horgan B, Simon L
… +33 more, Ioannidis JPA, Goel N, Tugwell P, Balay-Dustrude E, Bartlett SJ, Bingham CO, Boers M, Bouillot C, Böhm P, Choy E, Cowern M, Dagsvik K, Djurtoft C, Furst DE, Haugegaard T, Hazlewood GS, Hoens AM, Jørgensen TS, Kelly SE, Kruzikas D, Musaus J, Richards P, Shea BJ, Strand V, Thomas M, Torralba K, Voshaar M, Weinbrecht-Mischkewitz M, Woodworth TG, Wright G, Flurey C, Christensen R, OMERACT Safety Working Group
OBJECTIVES: To build on existing evidence regarding single-item measurement instruments of patient-reported bother or trouble from medical side effects in individuals with rheumatic and musculoskeletal diseases (RMDs). F...OBJECTIVES: To build on existing evidence regarding single-item measurement instruments of patient-reported bother or trouble from medical side effects in individuals with rheumatic and musculoskeletal diseases (RMDs). Further, to collect input from the OMERACT community through a structured survey that rated and ranked available options and to seek agreement to advance one or more of these measures for use as exploratory outcomes in future clinical trials. METHODS: At OMERACT 2025 we presented and discussed survey results for domain match, feasibility and ranking of six candidate instruments of bother or trouble from side effects. Collaborator feedback - including comments from patients, clinicians, and researchers - was synthesized with a large-language-model (LLM) to identify key concerns and guide refinement of the instrument's relevance, clarity, and acceptability. The LLM-assisted synthesis of participant comments resulted in a new, single-item instrument designed to improve patient safety reporting from the patient's perspective. RESULTS: The merged and modified version of the instrument was presented at the OMERACT 2025 meeting, where 33 participants approved it as a reasonable approach to incorporate collaborator input. The proposed instrument is feasible (32 [97%]) and voting supported advancing its further assessment (30 [91%]) as an exploratory outcome measurement instrument in coming RMD trials. CONCLUSION: We developed a novel single-item instrument. This is the first known application of LLMs in refining a patient-reported outcome instrument for clinical trials. It is designed to capture the patient perspective on symptomatic treatment-related side effects in RMDs and is supported for exploratory use in trials.
OBJECTIVES: To evaluate whether early canakinumab initiation may provide treatment advantages in Still's disease (SD) patients, particularly in terms of therapy discontinuation due to long-term disease remission, glucoco...OBJECTIVES: To evaluate whether early canakinumab initiation may provide treatment advantages in Still's disease (SD) patients, particularly in terms of therapy discontinuation due to long-term disease remission, glucocorticoid sparing effect, and increase in the frequency of monocyclic disease course rather than a polycyclic or chronic articular pattern. METHODS: SD patients treated with canakinumab were grouped according to time between disease onset and canakinumab initiation (≤3 months vs. >3 months). Patients were enrolled from the international AutoInflammatory Disease Alliance (AIDA) Network registry for SD. RESULTS: Overall, 190 patients were enrolled, 35 (19%) treated with canakinumab within three months from SD onset and 155 (82%) starting canakinumab later. Glucocorticoids use decreased more rapidly in patients receiving canakinumab within 3 months from SD onset than among patients treated later, with reductions of 50% vs 6% at month 3 (p=0.0001), and 75% vs 32% at month 6 (p=0.004). In logistic regression analysis, canakinumab initiation within 3 months from disease onset was significantly associated with treatment discontinuation due to long-term remission (OR 4.83, 95% CI 1.08-23.19; p=0.04). A monocyclic course occurred in 49% of patients starting canakinumab ≤3 months versus 8% starting later (p<0.0001). Starting canakinumab within 3 months from disease onset was significantly associated with a monocyclic disease course compared with the chronic-articular (RRR 4.43, 95% CI 1.12-17.60; p=0.034) and polycyclic courses (RRR 8.97, 95% CI 1.29-62.3; p=0.03). CONCLUSIONS: Early canakinumab initiation is associated with treatment discontinuation due to long-term remission and appears linked to a greater frequency of a monocyclic disease course.
BACKGROUND: Much of the evidence for a high prevalence of diabetes mellitus in dermatomyositis comes from studies of juvenile-onset dermatomyositis patients. Also, glucocorticoid therapy may confound diabetes risk. Our s...BACKGROUND: Much of the evidence for a high prevalence of diabetes mellitus in dermatomyositis comes from studies of juvenile-onset dermatomyositis patients. Also, glucocorticoid therapy may confound diabetes risk. Our study evaluated the prevalence of diabetes in adult-onset dermatomyositis, independent of glucocorticoid exposure, identified associated risk factors, and compared diabetes prevalence with that of a matched control population. METHODS: Our institutional database was queried to identify patients with adult-onset dermatomyositis using ICD-9 and ICD-10 codes over a 15-year study period (January 1, 2007 - December 31, 2021). Retrospective chart review identified diabetes using American Diabetes Association criteria and assessed the temporal relationship among adult-onset dermatomyositis diagnosis, diabetes onset, and glucocorticoid initiation. A contemporaneous non-dermatomyositis control cohort was identified. Propensity score matching at a 1:2 ratio was performed based on age, sex, body mass index, race, smoking status, hypertension, and dyslipidemia. Diabetes risk factors were identified using logistic regression. RESULTS: The adult-onset dermatomyositis cohort included 434 patients with a median age of 64 years. Diabetes was present in 23.6% of adult-onset dermatomyositis patients compared with 10.5% of controls (p < 0.001). Over half of diabetes diagnoses occurred before glucocorticoid initiation. Among patients with adult-onset dermatomyositis, higher body mass index, hypertension, and dyslipidemia were significantly associated with diabetes, whereas myositis-specific and associated antibodies were not. Adult-onset dermatomyositis was independently associated with increased odds of diabetes compared with matched controls (odds ratio 1.06; p = 0.008). CONCLUSION: Adult-onset dermatomyositis is associated with a significantly higher prevalence of diabetes independent of glucocorticoid exposure, supporting the need for early cardiometabolic screening in this population. FUNDING: None.
OBJECTIVES: Given the active role of the JAK signaling pathway and the multifactorial immune mediators observed in Behçet syndrome (BS), JAK inhibitors (JAKi) may be promising agents. In this systematic literature review...OBJECTIVES: Given the active role of the JAK signaling pathway and the multifactorial immune mediators observed in Behçet syndrome (BS), JAK inhibitors (JAKi) may be promising agents. In this systematic literature review (SLR), we aim to evaluate the efficacy and safety of JAKi in BS. METHODS: A systematic literature search was conducted in Embase, PubMed, and the Cochrane Library to identify all reports on the efficacy and safety of JAKi in patients with BS. The SLR protocol was registered with the International Prospective Registry of Systematic Reviews (CRD420251000172). We additionally presented our patient with gastrointestinal involvement treated with upadacitinib. RESULTS: Among the 92 patients (48 men, mean (SD) age 38.5 (13.5) years), 46 used tofacitinib, 31 used baricitinib, and 15 used upadacitinib. All but 1 patient had used at least one previous conventional immunosuppressive, and 57.6% were refractory to at least one prior tumor necrosis factor inhibitor. JAKi were used in combination with conventional immunosuppressive and/or glucocorticoids in 90.6% of the patients. The primary indication for JAKi initiation was gastrointestinal involvement in 46 (50%) patients including our patient, vascular involvement in 22 (23.9%), uveitis in 19 (20.6%), and mucocutaneous and/or articular involvement in 5 (5.6%) patients. The remission rates for overall, gastrointestinal, vascular, and eye involvement were 85.9%, 82.6%, 90.9%, and 84.2%. Serious adverse events were observed in 5.4% of the patients. No thrombotic events were reported. CONCLUSION: This SLR showed that JAKi may be effective in refractory BS patients with a favorable safety profile.
BACKGROUND/OBJECTIVES: Imaging is central to the diagnosis of giant cell arteritis (GCA). However, direct comparisons between magnetic resonance imaging (MRI) and ultrasound remain limited, particularly regarding vascula...BACKGROUND/OBJECTIVES: Imaging is central to the diagnosis of giant cell arteritis (GCA). However, direct comparisons between magnetic resonance imaging (MRI) and ultrasound remain limited, particularly regarding vascular involvement patterns and their combined diagnostic value. This study compared MRI and ultrasound in suspected GCA and evaluated whether their combination improves diagnostic accuracy. METHODS: We retrospectively analyzed 183 patients with suspected GCA evaluated at a tertiary care center between July 2017 and October 2022. MRI was performed in all patients using a graded sum score across multiple cranial and extracranial vessel territories. Ultrasound was performed in 159 patients (87 %) using a binary sum score assessing temporal, common carotid, and subclavian/axillary arteries. Vessel inflammation patterns were characterized across both modalities, and diagnostic performance was assessed using receiver operating characteristic curve analysis. RESULTS: GCA was diagnosed in 101 patients (55 %). Bilateral vessel involvement distinguished GCA from non-GCA and extended beyond subclavian/axillary arteries to include temporal, occipital, and vertebral territories. MRI achieved strong diagnostic accuracy (AUC 0.90; sensitivity 0.88; specificity 0.85), as did ultrasound (AUC 0.89; sensitivity 0.84; specificity 0.92). Cross-modality correlations were strongest for temporal arteries (r = 0.52). When MRI and ultrasound findings were concordant (82 % of cases), diagnostic performance improved further (AUC 0.94; sensitivity 0.96; specificity 0.92). CONCLUSION: MRI and ultrasound each provide high diagnostic accuracy for GCA while capturing complementary patterns of vascular inflammation. Bilateral involvement across multiple vascular territories is a defining disease feature. Concordant multimodal imaging substantially enhances diagnostic confidence and supports a combined imaging approach in patients with suspected GCA.
Lage-Hansen PR, Haugegaard T, Sofíudóttir BK
… +9 more, Svendsen N, Kirkham JJ, Amris K, de Wit M, Boers M, Kragsnaes MS, Choy E, Ellingsen T, Christensen R
OBJECTIVE: To assess agreement between physician and patient global assessment improvements in randomised controlled trials (RCT) of targeted therapies for rheumatoid arthritis and to identify which core outcome domains...OBJECTIVE: To assess agreement between physician and patient global assessment improvements in randomised controlled trials (RCT) of targeted therapies for rheumatoid arthritis and to identify which core outcome domains best explain changes in each assessment and their discrepancies. METHODS: We conducted a systematic review and meta-epidemiological analysis of RCTs comparing targeted therapies with placebo. Standardised mean differences (SMDs) were calculated for all eight RA-COS domains: Physician global assessment, Patient global assessment, Disability, Tender joint count (TJC), Swollen joint count (SJC), Pain, Acute phase reactants, and Fatigue. Agreement between physician and patient assessments was assessed as ΔSMD (i.e., SMD - SMD ). Multivariate meta-analyses were performed to calculate SMD for the remaining domains. Multiple imputation followed by meta-regression analyses identified domain-level predictors of global assessment changes. RESULTS: We identified 73 double-blind RCTs (165 randomised comparisons) involving 33,956 RA patients. Physician assessments demonstrated slightly greater treatment effects (SMD -0.60; 95% CI -0.65 to -0.55) compared with patient assessments (SMD -0.50; 95% CI -0.54 to -0.46): ΔSMD = -0.09 (95% CI -0.12 to -0.05). Univariable meta-regression identified disability, TJC and SJC as the strongest associations of both assessments, yet SJC was the only domain significantly associated with the contrast between these (ΔSMD). Pain was independently associated only with patient global assessment. CONCLUSION: Targeted therapies show larger improvements in physician-assessed outcomes than on patient-reported outcomes, largely driven by SJC. Pain remains a distinct determinant of patient assessments, underscoring the need to better integrate patient-reported outcomes into RA trial design. REGISTRATION: The protocol was registered on PROSPERO (2025-02-14). REGISTRATION NUMBER: CRD420250652342.
Teoh WS, Raghupathy J, Kanneganti A
… +8 more, Kadir HA, Teng GG, Tong PSY, Host LV, Morrisroe K, Nikpour M, Low AHL, Contributing Members of the SCTC Cancer Working Group
OBJECTIVE: SSc is associated with increased malignancy risk, a growing cause of mortality in SSc. However, standardised incidence ratios (SIR) vary widely across studies and different malignancy subtypes. This meta-analy...OBJECTIVE: SSc is associated with increased malignancy risk, a growing cause of mortality in SSc. However, standardised incidence ratios (SIR) vary widely across studies and different malignancy subtypes. This meta-analysis aimed to determine the SIR of different malignancy subtypes and the temporal relationship with SSc diagnosis. METHODS: A systematic review and meta-analysis were conducted by searching Embase, MEDLINE and Cochrane Library databases from inception to 7 December 2024. Cohort studies reporting SIRs of malignancies in SSc patients were included. Studies where SIR was not reported were excluded. Random-effects models were used to calculate pooled estimates, while study quality was assessed using the Newcastle-Ottawa Scale. This review is registered on PROSPERO (CRD42023445876). RESULTS: Thirty-one cohort studies comprising at least 32,134 SSc patients were included. Overall malignancy risk was significantly increased (SIR 1.66; 95% CI 1.32 - 2.08). Highest risks were observed for esophageal, liver, cervical, lung, and haematological malignancies (SIR 3.35 - 13.95), while breast malignancy showed modest increased risk (SIR 1.34, 95% CI 1.00 - 1.80) that was not statistically significant. High-quality studies confirmed these associations except for esophageal malignancy, with only one high quality study for liver malignancy. There was a mean interval of 7.4 years (95% CI 5.3 - 9.5) between SSc and malignancy diagnosis. CONCLUSION: Our findings confirm an increased malignancy risk, particularly lung, haematological and cervical malignancies, and an approximate 7-year interval between SSc diagnosis and malignancy development. Further studies are needed to clarify the risk factors for different malignancy subtypes to develop cancer screening strategies. FUNDING: NIL.
OBJECTIVE: To validate the reliability and feasibility of AI-automated grid placement for bone marrow lesion (BML) scoring in the Knee Inflammation MRI Scoring System (KIMRISS) using the OMERACT Filter. METHODS: Eleven e...OBJECTIVE: To validate the reliability and feasibility of AI-automated grid placement for bone marrow lesion (BML) scoring in the Knee Inflammation MRI Scoring System (KIMRISS) using the OMERACT Filter. METHODS: Eleven experts evaluated 40 MRI cases using manual and automated grid placement. Grids were compared both directly using spatial similarity metrics and indirectly using agreement metrics calculated on resulting KIMRISS BML scores. Feasibility was assessed using the System Usability Scale (SUS). RESULTS: In most regions, automatically- and manually-placed grids demonstrated strong spatial similarity (e.g., mean femur Dice Coefficient = 0.78) and KIMRISS BML score agreement (mean intraclass correlation coefficients of 0.86 and 0.89 for baseline and change scores, respectively). SUS scores for automated grid placement were moderate (mean = 66.1). CONCLUSION: Automated grid placement is a reliable and feasible improvement to KIMRISS that could improve the ease and reproducibility of quantifying osteoarthritis in clinical trials.
Terslev L, Schmidt NS, Fana V
… +20 more, Inanc N, DiRenzo D, Gordon RA, Magni-Manzoni S, Mar D, Misaki K, Miguel M, Möller I, Rizzo C, Blasi J, Coronel L, Sahbudin I, Todorov P, Torralba KD, Wakefield RJ, Mandl P, Pineda C, Keen H, D'Agostino MA, on the behalf of the OMERACT Ultrasound working group
OBJECTIVE: This study tested the reliability of an ultrasound scoring system (0-3) for lacrimal glands using an OMERACT semi-quantitative ultrasound scoring system developed for salivary glands in primary Sjögrens Diseas...OBJECTIVE: This study tested the reliability of an ultrasound scoring system (0-3) for lacrimal glands using an OMERACT semi-quantitative ultrasound scoring system developed for salivary glands in primary Sjögrens Disease pSjD). METHODS: Fourteen rheumatologists participated in a static image exercise on ultrasound images in order to evaluate the reliability of the existing salivary gland ultrasound (SGUS) scoring system when applied to lacrimal glands. All images were obtained from patients with suspected pSjD using a 6-24 MHz linear hockey stick transducer. One hundred images representing different grades of parenchyma changes in the lacrimal glands were collected for the inter-observer analyses and 32 of these were repeated to assess intra-observer reliability. Kappa statistics were applied. RESULTS: Participants had musculoskeletal ultrasound experience ranging from 2-28 years, and SGUS experience from 0-12 years. The majority (11/14) had no prior experience in lacrimal gland ultrasound but 13/14 had experience in SGUS. Intra-observer reliability was good to excellent, with kappa values ranging from 0.63 to 1.00 (95% CI: 0.77-0.89). Inter-observer reliability showed good agreement, with a kappa of 0.63 (95% CI: 0.61-0.65). CONCLUSION: The consensus-based semi-quantitative ultrasound scoring system for salivary glands showed good to excellent intra-observer and good inter-observer reliability when applied to the lacrimal glands using static images. The next step is to assess the reliability when scoring lacrimal glands in video-clips and in live exercises.