Background/Objective: Down syndrome-associated arthritis (DA) is an underrecognized, inflammatory arthritis that occurs in individuals with Down syndrome. The clinical course, optimal treatment and outcomes are currently unclear. The objective of this study was to identify the clinical presentation and longitudinal outcomes of DA compared with those of juvenile idiopathic arthritis (JIA) using the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) registry. Methods: A retrospective case-control study design matched patients with DA to those with JIA on age, gender, arthritis subtype, and medication exposure. Groups were compared at multiple visits (clinical and patient-reported outcomes). Mann-Whitney U test, Fisher’s exact test, and generalized linear mixed-effect models were used to compare outcomes between groups. Results: Twenty patients with DA were matched to 100 with JIA. The average intervals between the first and last visits were 3.2 years and 4.6 years for those with JIA and DA, respectively. Most had polyarticular arthritis (70%), while those with DA had more comorbid autoimmune conditions (20% vs. 0%), but no uveitis (0% versus 4%). At the last visit, those with DA had lower arthritis disease activity scores compared with those with JIA [1.8 (2.5) vs. 3.9 (4.2); p= 0.02]. The DA group had pain scores that improved over time and trended with disease activity scores. The JIA group had pain scores that increased over time and did not trend with disease activity scores. Conclusions: With similar disease severity and initial treatment, patients with DA have improved clinical outcomes over time with less active arthritis and reported pain compared with those with JIA.
BACKGROUND/OBJECTIVE:Adherence to treatment in axial spondyloarthritis (axSpA) remains poorly documented, with estimates ranging from 28.2% to 70.6%. Psychological factors may influence adherence. This study aimed to assess adherence to biological disease-modifying antirheumatic drugs (bDMARDs) and evaluate the impact of catastrophizing, fibromyalgia, anxiety, and depression on adherence. METHODS:We conducted a 1-year multicenter cross-sectional study, including consecutive axSpA patients fulfilling ASAS criteria. Participants completed a self-administered questionnaire covering demographic data, disease characteristics, and activity scores. Adherence was assessed using the Girerd scale (GS); psychological assessments included the Pain Catastrophizing Scale (PCS), Fibromyalgia Rapid Screening Tool (FIRST), and Hospital Anxiety and Depression Scale (HADS). RESULTS:Among 500 patients, 49.2% showed good adherence (GS=0). Prevalence of catastrophizing, fibromyalgia, anxiety, and depression was 17.2%, 21.4%, 25.6%, and 11.0%, respectively. Good adherence was significantly associated with older age (mean 53.3 vs. 45.9 y; p<0.001), retirement status (26.4% vs. 12.6%; p<0.001), and lower educational level (OR: 1.84; 95% CI: 1.23-2.75; p=0.003). Poor adherence was linked to anxiety (OR: 1.53; 95% CI: 1.02-2.30; p=0.041) and catastrophizing (OR: 1.61; 95% CI: 1.00-2.58; P=0.049). In multivariate analysis, younger age (OR: 0.96; 95% CI: 0.95-0.98; p<0.001) and anxiety (OR 1.72; 95% CI: 1.05-2.81; p=0.031) remained significantly associated with poor adherence. CONCLUSIONS:About half of axSpA patients had suboptimal adherence to bDMARDs. Younger age, anxiety, and catastrophizing were associated with poor adherence and should be considered in patient management.
OBJECTIVE:Children with systemic rheumatologic diseases (SRD) and autoinflammatory diseases can experience life-threatening deterioration requiring intensive care. We conducted a retrospective cohort study with 2 complementary components: (1) a descriptive analysis of clinical presentations and outcomes; and (2) an exploratory derivation of a bedside risk-stratification tool, intended as a candidate severity overlay on established pediatric intensive care unit (PICU) scoring systems rather than as a fully validated general-purpose prognostic model. DESIGN:Retrospective observational cohort study with exploratory prognostic model development. Propensity-score matching was used as a secondary, descriptive analysis to contextualize outcomes against comparable populations and is not intended to support causal inference. SETTING:Single academic pediatric intensive care unit (January 2005 to December 2015). PATIENTS:Forty children with confirmed SRD requiring PICU admission (first admission per patient) and propensity-matched controls (2:1 ratio). MEASUREMENTS AND MAIN RESULTS:Nearly two-thirds (62.2%) of patients experienced their first disease manifestation as a life-threatening crisis. Hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) was associated with the majority of deaths, accounting for 83.3% of fatalities despite representing only 20% of admissions (disease-specific mortality 62.5% vs. 6.3% for other diagnoses combined, p<0.001). Backward stepwise logistic regression identified 3 predictors that were combined, with equal weighting, into the exploratory PHV Score (PRISM-HLH-Vasoactive): Pediatric Risk of Mortality III (PRISM-III) at 24 hours >20, HLH/MAS diagnosis, and requirement for ≥2 vasoactive agents. In this derivation sample the score showed encouraging discrimination [apparent area under the curve (AUC) 0.91, 95% CI 0.83-0.99; optimism-corrected AUC 0.88]; however, given the very limited event count (n=6 deaths), wide CIs, and the fact that the PHV Score partially incorporates PRISM-III, head-to-head AUC comparisons with PRISM-III (AUC 0.72), PELOD-2 (Pediatric Logistic Organ Dysfunction-2; AUC 0.68), and pediatric SOFA (Sequential Organ Failure Assessment; AUC 0.64) should be interpreted with caution and regarded as hypothesis-generating. At the optimal cutoff of ≥2 (Youden index), sensitivity was 83.3% and specificity 91.2%; predictive values are prevalence-dependent and may not generalize to centers with different case mixes. CONCLUSIONS:In this retrospective single-center cohort, HLH/MAS was associated with most PICU deaths among children with systemic rheumatologic and autoinflammatory diseases. The PHV Score is an exploratory, candidate bedside tool that appears to refine risk stratification in our cohort but requires prospective multicenter external validation.
OBJECTIVE:Cognitive intrusion of pain, autobiographical memory, and aggression have not been simultaneously compared in rheumatic diseases. We compared these constructs across rheumatoid arthritis (RA), psoriatic arthritis (PsA), osteoarthritis (OA), and fibromyalgia (FM) and identified disease-specific predictors. METHODS:Two hundred twenty-five patients (62 RA, 52 PsA, 56 OA, 55 FM) were assessed (May to October 2025) with the Experience of Cognitive Intrusion of Pain Scale (ECIPS), Autobiographical Memory Questionnaire (AMQ), Buss-Perry Aggression Scale (BPAS), Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), and Visual Analog Scale (VAS). Disease burden was measured with the Disease Activity Score 28-C-reactive protein (DAS28-CRP), Disease Activity Index for Psoriatic Arthritis (DAPSA), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), and Fibromyalgia Impact Questionnaire (FIQ). Analysis of variance and covariance (ANOVA, ANCOVA; age, sex, depression, anxiety) and multiple regression were applied with Benjamini-Hochberg false discovery rate (FDR) correction. RESULTS:After adjustment, ECIPS did not differ across groups (F3,217=0.63, p=0.595, partial η²=0.009). Hostility differences attenuated after mood adjustment (p=0.784). Scene imagery differed among groups (p=0.023). Physical aggression (β=0.270, p=0.005) and memory rehearsal (β=0.653, p<0.001) were associated with cognitive intrusion. DAS28-CRP predicted ECIPS in RA (β=0.357, p=0.006) and FIQ in FM (β=0.401, p=0.003). No medication subgroup differences emerged. CONCLUSIONS:Cognitive pain intrusion did not differ across rheumatic diseases after mood adjustment (partial η²=0.009). Because the ECIPS lacks rheumatology validation, this may reflect limited measurement sensitivity, residual confounding, or insufficient power rather than true equivalence. Disease activity was independently associated with cognitive intrusion in RA and FM. Rheumatology-specific psychometric validation is warranted.
Objectives: Elicit patient feedback on a computerized systemic lupus erythematosus (SLE) patient decision aid (PtDA). Methods: We embedded a mixed-methods study in a multicenter SLE PtDA implementation project at 15 US rheumatology clinics during 2019-2023. A patient self-administered survey included questions on the acceptability of information quality (6-item), the amount of information, the inclusion of enough information for decision-making, the usefulness of the SLE PtDA in decision-making, and whether it was balanced (1-item each). Qualitative assessment was with 2 open-ended questions: (1) What did you like about the decision aid? (2) What suggestions do you have to improve the decision aid? Results: A high proportion of 1895 SLE patients, 78% to 92%, rated the acceptability of the information quality of SLE PtDA as good to excellent. Of participants, 82% rated the amount of information as just right; 79% reported inclusion of enough information; 90% found it useful for making SLE therapy decisions; and the presentation of the data in the SLE PtDA was balanced for 82%. The top 3 responses about what participants liked were: (1) SLE PtDA content (39.2%), (2) positive experience (36.6%), and (3) its format and design (9.0%). The top 3 suggestions about improving the SLE PtDA were: (1) provide information about treatment options and medication (14.7%), (2) improve the design (13.6%), and (3) provide more information (5.7%). Conclusions: The SLE PtDA met patients’ expectations for information amount and quality in a mixed-methods study. SLE PtDA was modified based on target population feedback.
Background/Objective: Juvenile dermatomyositis (JDM) is a systemic autoimmune vasculopathy, which may be complicated by calcinosis of the skin and subcutaneous tissues. Calcinosis is often associated with pain, ulceration, infection, impaired mobility, and reduced quality of life, yet no gold standard exists for its detection and longitudinal monitoring. Current evaluation relies on clinical examination (history plus physical examination) with or without targeted conventional radiography, which may underestimate disease burden and expose children to cumulative doses of ionizing radiation. The EOS 2D/3D imaging system provides rapid, whole-body imaging with substantially reduced radiation exposure. Thus, we sought to explore its utility in assessing calcinosis in JDM. Methods: In this retrospective case series, we investigated NYU pediatric patients with JDM who underwent EOS imaging for evaluation of calcinosis. EOS images and conventional radiographs were independently reviewed by 2 radiologists blinded to clinical data, with a focus on the anatomic distribution of calcinosis. Results: Seven patients (5 female, 2 male, ages 10 to 17 years) met the inclusion criteria, of whom 6 underwent both EOS and x-ray imaging. EOS imaging accurately identified calcinosis of the trunk and lower extremities in all cases and detected calcinosis not previously appreciated on clinical examination or dedicated radiographs in every patient. In 2 patients, EOS imaging failed to detect all upper-extremity calcinosis, likely due to the use of standard orthopedic positioning. Conclusions: EOS imaging appears to be a valid alternative to conventional radiography for evaluating calcinosis of the trunk and lower extremities in JDM, while offering the advantages of lower radiation exposure, rapid acquisition, and broader anatomic coverage. Development of JDM-specific positioning protocols may improve the detection of upper-extremity disease.
INTRODUCTION:Differentiating between infection and flare in patients with systemic lupus erythematosus (SLE) presents a clinical challenge due to the overlap of clinical manifestations and laboratory findings. The neutrophil-to-lymphocyte ratio (NLR) has been proposed as an accessible marker of systemic inflammation. The aim was to evaluate the association and diagnostic performance of NLR in identifying infection and differentiating it from a flare in patients with SLE. METHODS:A longitudinal analytical observational study was conducted at the Víctor Lazarte Echegaray Hospital (Peru) between January 2019 and June 2025. 213 patients with SLE were included, of whom 136 presented with flares and 77 with infection. The NLR was calculated from the complete blood count at the time of clinical evaluation. Its association with infection was assessed using logistic regression, and its diagnostic performance was evaluated using receiver operating characteristic ROC curves, comparing it with C-reactive protein (CRP). RESULTS:The NLR was significantly higher in patients with infection. In logistic regression analysis, elevated NLR values were associated with infection (OR: 1.25; 95% CI: 1.15-1.36; p<0.001), remaining significant after adjustment (adjusted OR: 1.18; 95% CI: 1.08-1.30; p<0.001). The NLR showed excellent discriminative ability (AUC: 0.97), with a cutoff point of 6.8, sensitivity of 90%, and specificity of 92%. C-reactive protein (CRP) showed similar performance (AUC: 0.97). The combined model increased sensitivity to 97% and reduced the negative likelihood ratio to 0.03. CONCLUSIONS:NLR is a simple, accessible, and low-cost marker that allows differentiation between infection and flare in patients with SLE, with diagnostic performance comparable to that of CRP. Its combined use can improve clinical decision-making in hospital settings.
BACKGROUND/OBJECTIVE:Systemic lupus erythematosus (SLE) is a known risk factor for femoral head osteonecrosis (FHON), but its impact on FHON risk following hip trauma has not been well characterized. We evaluated whether adults with SLE have an increased risk of FHON after hip trauma compared with patients without SLE. METHODS:We conducted a retrospective cohort study using de-identified electronic health record data from the TriNetX Research Network. Adults aged 18 to 65 years with an incident hip trauma diagnosis and no prior FHON were included. SLE was defined by the presence of an ICD-10 diagnosis code for SLE documented in the electronic health record. Patients with and without SLE were matched 1:1 using propensity scores incorporating demographics, baseline comorbidities, and systemic glucocorticoid exposure. The primary outcome was incident FHON within 5 years following hip trauma, with a secondary analysis evaluating FHON risk in matched cohorts without hip trauma. Kaplan-Meier and Cox proportional hazards analyses were performed. RESULTS:Among adults with incident hip trauma, patients with SLE had a higher 5-year incidence of FHON than patients without SLE. In propensity score-matched time-to-event analyses, SLE was associated with an increased hazard of osteonecrosis (hazard ratio: 2.9; 95% CI: 2.0-4.3; log-rank p < 0.001). These findings were consistent with a similarly elevated baseline risk of FHON observed in patients with SLE in analyses without hip trauma. CONCLUSIONS:SLE is associated with an increased FHON risk both with and without hip trauma. The modest absolute post-trauma risk difference supports symptom-guided vigilance rather than routine imaging after trauma. PRACTICAL POINT:Persistent, progressive, or atypical hip symptoms after trauma in patients with SLE warrant clinical vigilance for osteonecrosis, but these findings do not support routine imaging of all patients with SLE after trauma.
Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) is a leading cause of rapidly progressive glomerulonephritis and a major contributor to end-stage kidney disease (ESKD). Prognostic tools remain limited, particularly in Latin American populations. We historically analyzed 164 adults with biopsy-proven pauci-immune necrotizing and/or crescentic glomerulonephritis consistent with AAV, diagnosed between 2011 and 2024 at a tertiary referral center in Colombia. Clinical and histopathologic variables obtained near the kidney biopsy (≤30 d) were analyzed using Cox proportional hazards models. The primary outcome was ESKD, defined as initiation of chronic dialysis or sustained estimated glomerular filtration rate (eGFR) <15 mL/min/1.73 m². Independent predictors were used to derive the ANCLA (ANCA in Latin America) Risk Score. Model performance was evaluated using the area under the ROC curve (AUC), Harrell C-index, and Kaplan-Meier analysis. During a median follow-up of 12.9 months, 63 patients (38.4%) progressed to ESKD. Independent predictors included younger age, lower eGFR, higher 24-hour proteinuria, and a lower percentage of normal glomeruli. The ANCLA model demonstrated strong discrimination (AUC: 0.82, 95% CI: 0.79-0.85; C-index 0.72). Risk quartiles showed distinct kidney survival, with 2-year survival of ~95% in the lowest group versus <20% in the highest (log-rank p < 0.001). In comparative exploratory analyses, ANCLA outperformed the Berden classification and the Renal Risk Score within our cohort. The ANCLA Risk Score integrates routine clinical and histopathologic data into a simple, accurate tool for predicting kidney outcomes in AAV, including ANCA-negative cases (17.1%). Its strong performance in a Latin American cohort supports its potential for early risk stratification and clinical decision-making. External and multicenter validation are warranted.
BACKGROUND:Adolescents and young adults (AYAs) with rheumatic diseases (RD) may face challenges accessing and understanding information about methotrexate (MTX) and certain lifestyle choices, including drinking alcohol, using drugs, and sexual activity. We aimed to determine the knowledge, behaviours, and informational needs of AYAs taking MTX regarding alcohol/drug use and contraception. METHODS:AYAs 16 to 25 years old taking MTX completed a questionnaire codesigned by AYAs with RD. Questionnaires were accessed through rheumatology clinics and social media. RESULTS:Of 58 respondents, 78% were females, and 85% had JIA. In assessing knowledge of MTX, 28% were unaware that MTX can cause liver damage, and 87% agreed that alcohol should be avoided when taking MTX. The majority (80%) stated that contraception should be used if a female is taking MTX. Approximately one-quarter were unaware of the risk of birth defects while taking MTX. In assessing behaviours, 37% report drinking an average of 1 to 5 alcoholic drinks weekly. Sexual activity was reported by 58% of respondents, and almost all used contraception. Discomfort discussing alcohol use and sexual activity with their rheumatologist was reported by 20% and 32%, respectively. Respondents preferred to receive information from their rheumatologist/health care provider, the internet, and educational pamphlets. One-third reported not having access to accurate information regarding managing MTX side effects. CONCLUSION:AYAs often participate in at-risk behaviours while on MTX, which they do not discuss often with their rheumatologist. This study identified gaps and educational opportunities about MTX for AYAs, which can be shared through health care providers, online resources, or pamphlets.
A 31-year-old woman presenting with a total body rash and polyarthralgias for several weeks. Linear gingival erythema was uniquely noted on physical examination. Through testing, a diagnosis of SLE was made. Linear gingival erythema was uniquely noted on physical examination. Treatment was initiated with hydroxychloroquine, IV methylprednisolone, and topical clobetasol. Within days, the patient experienced rapid improvement in joint pain and oral ulcers.
OBJECTIVES:To determine the prevalence of attention deficit hyperactivity disorder (ADHD) in patients with fibromyalgia (FM) without a prior diagnosis of ADHD, compared with the general population, and to assess its relationship with FM impact. MATERIALS AND METHODS:A cross-sectional study was conducted in adults diagnosed with FM (ACR 2016 criteria) without known cognitive impairment, compared with age-matched, sex-matched, and education-matched controls. Participants completed the Fibromyalgia Impact Questionnaire (FIQ-R), the Argentine version of the Health Assessment Questionnaire (HAQ-A), the Montreal Cognitive Assessment (MoCA) for cognitive impairment, the Conners Continuous Performance Test II (CPT II) for adult ADHD, and the Wender-Utah Rating Scale (WURS) for retrospective childhood ADHD symptoms. Cognitive and neuropsychological tests were performed by specialized staff from the neurology service. Statistical analysis included univariate tests and multivariate logistic regression. RESULTS:Sixty FM patients and 71 controls were included. Adult ADHD was present in 61.7% of FM patients, with 48.6% undiagnosed in childhood. ADHD presence was not associated with higher FIQ-R or HAQ-A scores. Childhood ADHD symptoms were significantly associated with adult ADHD (OR: 62.3; p =0.003). FM patients had a higher prevalence of cognitive impairment (43.3% vs. 16.9%), childhood ADHD symptoms (31.7% vs. 14.1%), and adult ADHD (61.7% vs. 23.9%) compared with controls. CONCLUSIONS:Adult ADHD was highly prevalent in FM patients and often missed in childhood. Its presence was associated with prior childhood symptoms but not with greater disease impact or functional disability. FM patients also showed increased cognitive impairment and ADHD symptoms compared with the general population.
OBJECTIVE:To determine clinical factors associated with pulmonary hypertension (PH) and assess its impact on clinical outcomes in a predominantly Black and Hispanic systemic lupus erythematosus (SLE) cohort from an underserved US urban setting. METHODS:We conducted an observational cohort study of 140 adults with SLE treated at a large academic center in the Bronx, New York, from 2017 to 2021. PH was diagnosed by clinicians, supported by a right ventricular or pulmonary artery systolic pressure >35 mm Hg by echocardiography. The primary outcome was time to clinician-adjudicated lupus flare-related hospitalization. Secondary outcomes included hospitalization due to lupus flare with cardiac involvement and all-cause mortality. Cox proportional hazards models with PH as a time-varying covariate were used to assess associations. RESULTS:At baseline, the following comorbidities were all significantly more common in patients with PH compared with those without interstitial lung disease (ILD) (22.9% vs. 8.7%; p=0.03), Raynaud phenomenon (66.7% vs. 39.1%; p<0.01), atrial fibrillation (12.5% vs. 0%; p<0.01), and sepsis (20.8% vs. 7.6%; p=0.03). Over a median follow-up of 84 months, 57 patients (41%) experienced a flare-related hospitalization. In multivariable analysis, PH was an independent predictor of earlier lupus flare-related hospitalization (HR: 1.90; 95% CI: 1.07-3.38; p=0.03), as were younger age (HR: 0.97; 95% CI: 0.96-0.99; p<0.01) and higher SLEDAI scores (HR: 1.11; 95% CI: 1.05-1.18; p<0.01). PH was not significantly associated with cardiac-related hospitalization or all-cause mortality, though trends suggested higher event rates in the PH group. CONCLUSIONS:In this predominantly Black and Hispanic cohort with SLE, PH emerged as a clear, independent predictor of lupus flare-related hospitalizations. Vigilant PH screening and timely intervention may help reduce morbidity in high-risk, underserved SLE populations.
BACKGROUND:Rheumatoid arthritis is a disease whose treatment requires patient commitment to control disease activity, which is directly related to self-efficacy. The objective of this study was to map physical, psychological, social support, treatment adherence, and educational program variables associated with self-efficacy, as measured by validated RA-specific instruments. METHODS:This scoping review followed a comprehensive search of major biomedical databases and gray literature. Inclusion criteria were full-text articles, theses, and dissertations published from 1977 onwards, in English, Spanish, or Portuguese. The search identified 4737 records, of which 43 studies met the criteria and were included in the final sample. RESULTS:The studies originated from 5 continents and were published in English and Spanish, exhibiting heterogeneous characteristics. All used quantitative methods, with the majority being cross-sectional (44.19%, n=19), followed by randomized clinical trials (39.53%, n=17). Most participants were female (76.60%, n=5580), with a mean age of 55.90 years. The results were categorized into 3 areas: positive, negative, and nonsignificant associations. Treatment adherence, positive psychological factors, and social support were positively associated with self-efficacy, whereas physical factors and negative psychological factors showed negative associations. In the education and self-management group, 10 educational and self-management programs showed a positive association with self-efficacy. In contrast, 4 other programs showed no significant association. CONCLUSIONS:Although the results do not fully reflect causal relationships, they suggest the importance of addressing the psychological, physical, treatment adherence, and social support factors in individuals with rheumatoid arthritis, as well as promoting educational and self-management measures to increase self-efficacy.
