BACKGROUND: The ability to predict rheumatoid arthritis (RA) flares between clinic visits based on real-time, longitudinal patient-generated data could potentially allow for timely interventions to avoid disease worsening.
OBJECTIVE: This exploratory study aims to investigate the feasibility of using machine learning methods to classify self-reported RA flares based on a small data set of daily symptom data collected on a smartphone app.
METHODS: Daily symptoms and weekly flares reported on the Remote Monitoring of Rheumatoid Arthritis (REMORA) smartphone app from 20 patients with RA over 3 months were used. Predictors were several summary features of the daily symptom scores (eg, pain and fatigue) collected in the week leading up to the flare question. We fitted 3 binary classifiers: logistic regression with and without elastic net regularization, a random forest, and naive Bayes. Performance was evaluated according to the area under the curve (AUC) of the receiver operating characteristic curve. For the best-performing model, we considered sensitivity and specificity for different thresholds in order to illustrate different ways in which the predictive model could behave in a clinical setting.
RESULTS: The data comprised an average of 60.6 daily reports and 10.5 weekly reports per participant. Participants reported a median of 2 (IQR 0.75-4.25) flares each over a median follow-up time of 81 (IQR 79-82) days. AUCs were broadly similar between models, but logistic regression with elastic net regularization had the highest AUC of 0.82. At a cutoff requiring specificity to be 0.80, the corresponding sensitivity to detect flares was 0.60 for this model. The positive predictive value (PPV) in this population was 53%, and the negative predictive value (NPV) was 85%. Given the prevalence of flares, the best PPV achieved meant only around 2 of every 3 positive predictions were correct (PPV 0.65). By prioritizing a higher NPV, the model correctly predicted over 9 in every 10 non-flare weeks, but the accuracy of predicted flares fell to only 1 in 2 being correct (NPV and PPV of 0.92 and 0.51, respectively).
CONCLUSIONS: Predicting self-reported flares based on daily symptom scorings in the preceding week using machine learning methods was feasible. The observed predictive accuracy might improve as we obtain more data, and these exploratory results need to be validated in an external cohort. In the future, analysis of frequently collected patient-generated data may allow us to predict flares before they unfold, opening opportunities for just-in-time adaptative interventions. Depending on the nature and implication of an intervention, different cutoff values for an intervention decision need to be considered, as well as the level of predictive certainty required.

BACKGROUND: Early extubation (EEx) is defined as the removal of the endotracheal tube within 8 h postoperatively. The present study involved determining the availability and threshold of the vasoactive-inotropic score (VIS) for predicting EEx in adults after elective rheumatic heart valve surgery.
METHODS: The present study was designed as a single-center retrospective cohort study which was conducted with adults who underwent elective rheumatic heart valve surgery with CPB. The highest VIS in the immediate postoperative period was used in the present study. The primary outcome, the availability of VIS for EEx prediction and the optimal threshold value were determined using ROC curve analysis. The gray zone analysis of the VIS was performed by setting the false negative or positive rate R = 0.05, and the perioperative risk factors for prolonged EEx were identified by multivariate logistic analysis. The postoperative complications and outcomes were compared between different VIS groups.
RESULTS: Among the 409 patients initially screened, 379 patients were ultimately included in the study. The incidence of EEx was determined to be 112/379 (29.6%). The VIS had a good predictive value for EEx (AUC = 0.864, 95% CI: [0.828, 0.900], P < 0.001). The optimal VIS threshold for EEx prediction was 16.5, with a sensitivity of 71.54% (65.85-76.61%) and a specificity of 88.39% (81.15-93.09%). The upper and lower limits of the gray zone for the VIS were determined as (12, 17.2). The multivariate logistic analysis identified age (OR, 1.060; 95% CI: 1.017-1.106; P = 0.006), EF% (OR, 0.798; 95% CI: 0.742-0.859; P < 0.001), GFR (OR, 0.933; 95% CI: 0.906-0.961; P < 0.001), multiple valves surgery (OR, 4.587; 95% CI: 1.398-15.056; P = 0.012), and VIS > 16.5 (OR, 12.331; 95% CI: 5.015-30.318; P < 0.001) as the independent risk factors for the prolongation of EEx. The VIS ≤ 16.5 group presented a greater success rate for EEx, a shorter invasive ventilation support duration, and a lower incidence of complications than did the VIS > 16.5 group, while the incidence of reintubation was similar between the two groups.
CONCLUSIONS: In adults, after elective rheumatic heart valve surgery, the highest VIS in the immediate postoperative period was a good predictive value for EEx, with a threshold of 16.5.

UNASSIGNED: Osteoporosis is a common diagnosis and comorbidity observed in patients with rheumatic diseases. It is frequently associated with conditions such as rheumatoid arthritis, spondyloarthropathy, systemic lupus erythematosus, and other autoimmune rheumatic diseases.
UNASSIGNED: The incidence of osteoporosis is influenced by factors such as uncontrolled disease, prolonged and higher doses of steroid use, immobility, advanced age, and postmenopausal status. Achieving good control of the underlying disease, minimizing or avoiding the use of steroids for extended periods, and ensuring adequate supplementation of vitamin D and calcium are crucial in reducing the incidence of osteoporosis. Regular screening and appropriate management of osteoporosis can significantly decrease the associated morbidity and mortality.

UNASSIGNED: Social media data may augment understanding of the disease and treatment experiences and quality of life of youth with chronic medical conditions. Little is known about the willingness to share social media data for health research among youth with chronic medical conditions and the differences in health status between sharing and nonsharing youth with chronic medical conditions.
UNASSIGNED: We aimed to evaluate the associations between patient-reported measures of disease symptoms and functioning and the willingness to share social media data.
UNASSIGNED: Between February 2018 and August 2019, during routine clinic visits, survey data about social media use and the willingness to share social media data (dependent variable) were collected from adolescents in a national rheumatic disease registry. Survey data were analyzed with patient-reported measures of disease symptoms and functioning and a clinical measure of disease activity, which were collected through a parent study. We used descriptive statistics and multivariate logistic regression to compare patient-reported outcomes between youth with chronic medical conditions who opted to share social media data and those who did not opt to share such data.
UNASSIGNED: Among 112 youths, (age: mean 16.1, SD 1.6 y; female: n=72, 64.3%), 83 (74.1%) agreed to share social media data. Female participants were more likely to share (P=.04). In all, 49 (43.8%) and 28 (25%) participants viewed and posted about rheumatic disease, respectively. Compared to nonsharers, sharers reported lower mobility (T-score: mean 49.0, SD 9.4 vs mean 53.9, SD 8.9; P=.02) and more pain interference (T-score: mean 45.7, SD 8.8 vs mean 40.4, SD 8.0; P=.005), fatigue (T-score: mean 49.1, SD 11.0 vs mean 39.7, SD 9.7; P<.001), depression (T-score: mean 48.1, SD 8.9 vs mean 42.2, SD 8.4; P=.003), and anxiety (T-score: mean 45.2, SD 9.3 vs mean 38.5, SD 7.0; P<.001). In regression analyses adjusted for age, sex, study site, and Physician Global Assessment score, each 1-unit increase in symptoms was associated with greater odds of willingness to share social media data, for measures of pain interference (Adjusted Odds Ratio [AOR] 1.07, 95% CI 1.001-1.14), fatigue (AOR 1.08, 95% CI 1.03-1.13), depression (AOR 1.07, 95% CI 1.01-1.13), and anxiety (AOR 1.10, 95% CI 1.03-1.18).
UNASSIGNED: High percentages of youth with rheumatic diseases used and were willing to share their social media data for research. Sharers reported worse symptoms and functioning compared to those of nonsharers. Social media may offer a potent information source and engagement pathway for youth with rheumatic diseases, but differences between sharing and nonsharing youth merit consideration when designing studies and evaluating social media-derived findings.

OBJECTIVE: The aim of the present study was to illustrate the (potential) diagnostic role of high resolution US images in assessing the elementary lesions of dactylitis.
METHODS: Using high-frequency US machines/probes, we matched the micro-anatomical cadaveric architecture of the digit with multiple sonographic findings of dactylitis. High-sensitive color/power Doppler assessments have also been performed to evaluate the digital microvasculature.
CONCLUSIONS: Modern US equipment/features guarantee prompt and in-depth B-mode and color/power Doppler imaging of tiny anatomical structures of the digit which are usually not properly visible with standard US machines. More specifically, hypervascularization of the digital subcutaneous tissue, fibrous pulleys of flexor tendons, dorsal synovial pads as well as pathological changes of the distal entheseal anchorage network can be accurately detected.
CONCLUSIONS: In clinical practice, high-end US equipment can be used to accurately assess the digits in patients with dactylitis. This way, simple and convenient sonographic diagnosis of different elementary lesions can be timely established.

Cardiac magnetic resonance (CMR) is emerging as the modality of choice to assess early cardiovascular involvement in patients with autoimmune rheumatic diseases (ARDs) that often has a silent presentation and may lead to changes in management. Besides being reproducible and accurate for functional and volumetric assessment, the strength of CMR is its unique ability to perform myocardial tissue characterization that allows the identification of inflammation, edema, and fibrosis. Several CMR biomarkers may provide prognostic information on the severity and progression of cardiovascular involvement in patients with ARDs. In addition, CMR may add value in assessing treatment response and identification of cardiotoxicity related to therapy with immunomodulators that are commonly used to treat these conditions. In this review, we aim to discuss the following objectives: •Illustrate imaging findings of multi-parametric CMR approach in the diagnosis of cardiovascular involvement in various ARDs;•Review the CMR signatures for risk stratification, prognostication, and guiding treatment strategies in ARDs;•Describe the utility of routine and advanced CMR sequences in identifying cardiotoxicity related to immunomodulators and disease-modifying agents in ARDs;•Discuss the limitations of CMR, recent advances, current research gaps, and potential future developments in the field.

UNASSIGNED: Mitral regurgitation may develop or worsen following right ventricular apical pacing due to dyssynchronous left ventricular contraction. Pre-existing secondary mitral annular dilation is a well-recognized and important contributing factor. This description of pacing-induced torrential mitral regurgitation in the setting of rheumatic mitral valve disease is a rare case in which a primary mitral valve lesion was the antecedent mechanism.
UNASSIGNED: A 60-year-old man was admitted with dizziness and pre-syncope. Twelve-lead electrocardiogram showed complete heart block. A dual-chamber pacemaker was implanted and programmed in DDD mode. Transthoracic echocardiography performed a day later demonstrated a left ventricular ejection fraction (LVEF) of 63% and moderate mitral regurgitation. The patient presented 4 months later with breathlessness and orthopnoea. Pacemaker interrogation demonstrated a 98% right ventricular pacing burden. Echocardiography revealed torrential mitral regurgitation secondary to left ventricular dyssynchrony and complete loss of leaflet coaptation with preserved systolic function. Post-capillary pulmonary hypertension was diagnosed following right heart catheterization. The patient underwent metallic mitral valve replacement, tricuspid annuloplasty, and left internal mammary artery grafting to the left anterior descending artery for a severe proximal stenosis. On inspection, the native mitral valve was notably rheumatic in appearance, and this was confirmed histologically.
UNASSIGNED: It is important to closely monitor the progression of mitral regurgitation in those with primary mitral valve disease undergoing right ventricular pacing. Early follow-up may prevent the adverse haemodynamic consequences of worsening mitral regurgitation, with a greater chance of recovery of left ventricular function following surgery.

OBJECTIVE: Rheumatic diseases in children are chronic and multisystemic diseases. In this study, it was aimed to evaluate gastrointestinal endoscopic findings in children diagnosed as autoimmune or autoinflammatory rheumatic diseases consulted with pediatric gastroenterology for gastrointestinal complaints.
METHODS: The patients followed up by the Pediatric Rheumatology Department and consulted to the Pediatric Gastroenterology Department due to gastrointestinal complaints were included in the study. File records of the patients were analyzed retrospectively.
RESULTS: A total of 28 patients were included in the study. Twelve of the patients had autoimmune disease (Juvenile idiopathic arthritis [JIA], systemic lupus erythematosus, Sjögren\'s syndrome, and scleroderma) and the other 16 had autoinflammatory disease (familial Mediterrnean fever, hyper Immunoglobulin D syndrome, undifferantiated systemic autoinflammatory disease, and systemic JIA). Four of the patients with familial Mediterrnean fever also diagnosed as JIA. The mean age of the patients was 11.7±3.5 years. The main gastrointestinal complaints of patients with both autoimmune and autoinflammatory diseases were abdominal pain and diarrhea. Inflammatory bowel disease was found in 33% of those with autoimmune disease and 56% of those with autoinflammatory disease in patients underwent endoscopic evaluation. M694V mutation was present in 62% of the patients with autoinflammatory disease presented with gastrointestinal complaints.
CONCLUSIONS: Both autoimmune and autoinflammatory rheumatic diseases can cause gastrointestinal complaints and should be referred to a pediatric gastroenterologist for early diagnosis.

Children with paediatric rheumatic diseases (PRDs) are at increased risk of vaccine-preventable disease. Safe and effective vaccination is central to preventive care in PRD patients; however, uncertainty surrounding immunogenicity and safety has contributed to suboptimal vaccination. The aim of this study was to evaluate treatment effect on immunogenicity to vaccination in PRD patients and assess vaccine safety, specifically adverse events following immunisation (AEFI) and disease flare. Scoping review. In this scoping review, a systematic search of PubMed, CINAHL and Embase databases was conducted from 2014 to 23 August 2022 to identify observational studies evaluating the immunogenicity and safety of commonly used vaccinations in PRD patients. The primary outcome was immunogenicity (defined as seroprotection and protective antibody concentrations), with secondary outcomes describing AEFI and disease flare also extracted. Due to extensive heterogeneity related to diagnostic and vaccination variability, narrative synthesis was used to describe the findings of each study. Study quality was assessed via the Mixed Methods Appraisal Tool. The review was prospectively registered with PROSPERO (CRD42022307212). The search yielded 19 studies evaluating immunogenicity to vaccination and incidence of AEFI and disease flares in this population, which were of acceptable quality. Corticosteroids did not have deleterious effects on vaccine response. Treatment with conventional disease-modifying antirheumatic drugs (DMARDs) and biologic DMARDs generally had no effect immunogenicity in PRD patients. While patients exhibited adequate seroprotection, protective antibody levels were lower in patients on some immunosuppressant agents. Varicella infections were recorded post vaccination in several patients with low protective antibody levels undergoing treatment with DMARDs and corticosteroids. Most vaccines appear safe and effective in PRD patients, despite immunosuppressant treatment. Booster vaccinations should be considered with some studies highlighting inadequate seroprotection following primary course of vaccinations with acceleration of antibody decline over time. There was limited evidence to support avoiding live vaccines in PRD patients.

Cell-free (cf) extrachromosomal circular DNA (eccDNA) has a potential clinical application as a biomarker. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a complex immunological pathogenesis, associated with autoantibody synthesis. A previous study found that SLE patients with deoxyribonuclease 1-like 3 (DNASE1L3) deficiency exhibit changes in the frequency of short and long eccDNA in plasma compared to controls. Here, using the DifCir method for differential analysis of short-read sequenced purified eccDNA data based on the split-read signal of the eccDNA on circulomics data, we show that SLE patients with DNASE1L3 deficiency have a distinctive profile of eccDNA excised by gene regions compared to controls. Moreover, this profile is specific; cf-eccDNA from the top 93 genes is detected in all SLE with DNASE1L3 deficiency samples, and none in the control plasma. The top protein coding gene producing eccDNA-carrying gene fragments is the transcription factor BARX2, which is involved in skeletal muscle morphogenesis and connective tissue development. The top gene ontology terms are \'positive regulation of torc1 signaling\' and \'chondrocyte development\'. The top Harmonizome terms are \'lymphopenia\', \'metabolic syndrome x\', \'asthma\', \'cardiovascular system disease\', \'leukemia\', and \'immune system disease\'. Here, we show that gene associations of cf-eccDNA can serve as a biomarker in the autoimmune rheumatic diseases.