POS1394 ACCURACY AND PERFORMANCE OF A HANDHELD ULTRASOUND DEVICE TO ASSESS ARTICULAR AND PERIARTICULAR PATHOLOGIES IN PATIENTS WITH INFLAMMATORY ARTHRITIS

Background:Handheld ultrasound (HHUS) devices have increasingly found their way into clinical practice due to several advantages (e.g. portability, significantly lower purchase cost). However, there is no evidence to date on the accuracy and performance of HHUS in patients with inflammatory arthritis (IA).Objectives:To assess accuracy and performance of a new HHUS machine in comparison to a conventional cart-based sonographic machine in patients with IA.Methods:Consecutive IA patients of our outpatient clinic with at least one tender and swollen joint in the 66/68 joint count were enrolled. US was performed on clinically affected joints with corresponding tendons/entheses using a cart-based sonographic device ("Samsung HS40") and a HHUS device ("Butterfly iQ") in standard scan positions. One blinded reader scored all images for the presence of following pathologic findings: erosions, bony enlargement, synovial hyperthrophy, joint effusion, bursitis, tenosynovitis and enthesitis. In addition, synovitis was graded (B Mode and power Doppler (PD)) by the 4-level EULAR-OMERACT scale [1]. To avoid bias by the blinded reader, who otherwise would have been tempted to identify pathological findings for each examined joint, we also included 67 joints of two healthy volunteers into the evaluation. We calculated the overall concordance and the concordance by type of joint and type of pathological finding between the two devices (percentage of observation pairs in which the same rating was given by both devices). The Cohen's kappa coefficient (κ) with 95% bootstrap confidence intervals was used to assess the agreement between the two US devices. We also measured the time required for the US examination of one joint with both devices.Results:32 patients (20 rheumatoid arthritis, 10 psoriatic arthritis, 1 gouty arthritis, 1 systemic lupus erythematosus) were included in this study. Mean age of patients was 58.2±13.7 years, 63% were females. In total 186 joints were examined. The overall raw concordance in B-mode between the two devices was 97 %, with an overall κappa for agreement of 0.90, 95% CI (0.89, 0.94). No significant differences were found in relation to type of joint or pathological finding examined. The PD-mode of the HHUS device did not detect any PD-signal, whereas the cart-based device detected a PD-signal in 61 joints (33%). The portable device did not offer any time saving compared to the cart-based device (mean time in seconds per examined region: 47 seconds for the HHUS device versus 46.3 seconds for the cart-based device).Conclusion:The HHUS device "Butterfly iQ" has been shown to be accurate in the assessment of structural joint damage and inflammation in patients with IA, but only in B-mode. Significant improvements are still needed to reliable demonstrate blood flow detection by PD mode.References:[1]D'Agostino, M.A., et al., RMD Open, 2017. 3(1): p. e000428.Table 1.Concordance between a handheld and a conventional cart-based US device in B-modeAgreement by siteN joints (%)Concordance (%)Kappa 95%CIOverall186970.90 (0.89 to 0.94)Wrist32 (17.2)960.86 (0.77 to 0.93)Finger/toe joint (MCP, PIP, DIP, MTP)114 (61.3)970.92 (0.88 to 0.95)Elbows11 (5.9)950.87 (0.75 to 0.97)Shoulder4 (2.2)1001.00 (NA to NA) *Knee20 (10.7)980.96 (0.90 to 1.00)Ankle5 (2.7)1001.00 (NA to NA) *Agreement by pathological findingJoint effusion950.81 (0.68 to 0.92)Synovitis940.87 (0.79 to 0.93)Synovitis OMERACT grade (0– 3)900.84 (0.76 to 0.91)Bone enlargement980.88 (0.71 to 1.00)Erosion980.89 (0.77 to 0.89)Tenosynovitis980.83 (0.61 to 0.96)Entheseopathy1001.00 (NA to NA) *Bursitis970.90 (0.89 to 0.94)* unreliable kappa statistics because of small number of shoulders/ankles examined and small number of entheseopathiesFigure 1.Pathological US findings in MCP joints (1, 2, 3) and wrist (4) depicted by the two different ultrasound devicesB-mode erosive (arrow) and synovial (asterisk) changes could be detected by both devices (1-2), while PD changes of different grades only by the conventional US device (3-4).Acknowledgements:This study was supported by the Deutsche Forschungsgemeinschaft (DFG- FOR2886 PANDORA and the CRC1181). Additional funding was received by the Bundesministerium für Bildung und Forschung (BMBF; project METARTHROS, MASCARA), the H2020 GA 810316 - 4D-Nanoscope ERC Synergy Project, the IMI funded project RTCure, the Emerging Fields Initiative MIRACLE of the Friedrich-Alexander-Universität Erlangen-Nürnberg, the Else Kröner-Memorial Scholarship (DS, no. 2019_EKMS.27) and Innovationsfond Lehre / FAU Erlangen-Nürnberg 2019.Disclosure of Interests:None declare

Machine learning-based improvement of an online rheumatology referral and triage system

IntroductionRheport is an online rheumatology referral system allowing automatic appointment triaging of new rheumatology patient referrals according to the respective probability of an inflammatory rheumatic disease (IRD). Previous research reported that Rheport was well accepted among IRD patients. Its accuracy was, however, limited, currently being based on an expert-based weighted sum score. This study aimed to evaluate whether machine learning (ML) models could improve this limited accuracy.Materials and methodsData from a national rheumatology registry (RHADAR) was used to train and test nine different ML models to correctly classify IRD patients. Diagnostic performance was compared of ML models and the current algorithm was compared using the area under the receiver operating curve (AUROC). Feature importance was investigated using shapley additive explanation (SHAP).ResultsA complete data set of 2265 patients was used to train and test ML models. 30.5% of patients were diagnosed with an IRD, 69.3% were female. The diagnostic accuracy of the current Rheport algorithm (AUROC of 0.534) could be improved with all ML models, (AUROC ranging between 0.630 and 0.737). Targeting a sensitivity of 90%, the logistic regression model could double current specificity (17% vs. 33%). Finger joint pain, inflammatory marker levels, psoriasis, symptom duration and female sex were the five most important features of the best performing logistic regression model for IRD classification.ConclusionIn summary, ML could improve the accuracy of a currently used rheumatology online referral system. Including further laboratory parameters and enabling individual feature importance adaption could increase accuracy and lead to broader usage

Toward earlier diagnosis using combined eHealth tools in rheumatology: the joint pain assessment scoring tool (JPAST) project

Outcomes of patients with inflammatory rheumatic diseases have significantly improved over the last three decades, mainly due to therapeutic innovations, more timely treatment, and a recognition of the need to monitor response to treatment and to titrate treatments accordingly. Diagnostic delay remains a major challenge for all stakeholders. The combination of electronic health (eHealth) and serologic and genetic markers holds great promise to improve the current management of patients with inflammatory rheumatic diseases by speeding up access to appropriate care. The Joint Pain Assessment Scoring Tool (JPAST) project, funded by the European Union (EU) European Institute of Innovation and Technology (EIT) Health program, is a unique European project aiming to enable and accelerate personalized precision medicine for early treatment in rheumatology, ultimately also enabling prevention. The aim of the project is to facilitate these goals while at the same time, reducing cost for society and patients.Pathophysiology and treatment of rheumatic disease

Long-term safety of COVID vaccination in individuals with idiopathic inflammatory myopathies: results from the COVAD study

Limited evidence on long-term COVID-19 vaccine safety in patients with idiopathic inflammatory myopathies (IIMs) continues to contribute to vaccine hesitancy. We studied delayed-onset vaccine adverse events (AEs) in patients with IIMs, other systemic autoimmune and inflammatory disorders (SAIDs), and healthy controls (HCs), using data from the second COVID-19 Vaccination in Autoimmune Diseases (COVAD) study. A validated self-reporting e-survey was circulated by the COVAD study group (157 collaborators, 106 countries) from Feb-June 2022. We collected data on demographics, comorbidities, IIM/SAID details, COVID-19 history, and vaccination details. Delayed-onset (> 7 day) AEs were analyzed using regression models. A total of 15165 respondents undertook the survey, of whom 8759 responses from vaccinated individuals [median age 46 (35-58) years, 74.4% females, 45.4% Caucasians] were analyzed. Of these, 1390 (15.9%) had IIMs, 50.6% other SAIDs, and 33.5% HCs. Among IIMs, 16.3% and 10.2% patients reported minor and major AEs, respectively, and 0.72% (n = 10) required hospitalization. Notably patients with IIMs experienced fewer minor AEs than other SAIDs, though rashes were expectedly more than HCs [OR 4.0; 95% CI 2.2-7.0, p < 0.001]. IIM patients with active disease, overlap myositis, autoimmune comorbidities, and ChadOx1 nCOV-19 (Oxford/AstraZeneca) recipients reported AEs more often, while those with inclusion body myositis, and BNT162b2 (Pfizer) recipients reported fewer AEs. Vaccination is reassuringly safe in individuals with IIMs, with AEs, hospitalizations comparable to SAIDs, and largely limited to those with autoimmune multimorbidity and active disease. These observations may inform guidelines to identify high-risk patients warranting close monitoring in the post-vaccination period

High fatigue scores in patients with idiopathic inflammatory myopathies: a multigroup comparative study from the COVAD e-survey

Idiopathic inflammatory myopathies (IIMs) confer a significant risk of disability and poor quality of life, though fatigue, an important contributing factor, remains under-reported in these individuals. We aimed to compare and analyze differences in visual analog scale (VAS) scores (0–10&nbsp;cm) for fatigue (VAS-F) in patients with IIMs, non-IIM systemic autoimmune diseases (SAIDs), and healthy controls (HCs). We performed a cross-sectional analysis of the data from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) international patient self-reported e-survey. The COVAD survey was circulated from December 2020 to August 2021, and details including demographics, COVID-19 history, vaccination details, SAID details, global health, and functional status were collected from adult patients having received at least one COVID-19 vaccine dose. Fatigue experienced 1 week prior to survey completion was assessed using a single-item 10&nbsp;cm VAS. Determinants of fatigue were analyzed in regression models. Six thousand nine hundred and eighty-eight respondents (mean age 43.8&nbsp;years, 72% female; 55% White) were included in the analysis. The overall VAS-F score was 3 (IQR 1–6). Patients with IIMs had similar fatigue scores (5, IQR 3–7) to non-IIM SAIDs [5 (IQR 2–7)], but higher compared to HCs (2, IQR 1–5; P &lt; 0.001), regardless of disease activity. In adjusted analysis, higher VAS-F scores were seen in females (reference female; coefficient −0.17; 95%CI −0.21 to −13; P &lt; 0.001) and Caucasians (reference Caucasians; coefficient −0.22; 95%CI −0.30 to −0.14; P &lt; 0.001 for Asians and coefficient −0.08; 95%CI −0.13 to 0.30; P = 0.003 for Hispanics) in our cohort. Our study found that patients with IIMs exhibit considerable fatigue, similar to other SAIDs and higher than healthy individuals. Women and Caucasians experience greater fatigue scores, allowing identification of stratified groups for optimized multidisciplinary care and&nbsp;improve&nbsp;outcomes such as&nbsp;quality of life

Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern

Influence of antisynthetase antibodies specificities on antisynthetase syndrome clinical spectrum time course

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition

COVAD survey 2 long-term outcomes: unmet need and protocol

Vaccine hesitancy is considered a major barrier to achieving herd immunity against COVID-19. While multiple alternative and synergistic approaches including heterologous vaccination, booster doses, and antiviral drugs have been developed, equitable vaccine uptake remains the foremost strategy to manage pandemic. Although none of the currently approved vaccines are live-attenuated, several reports of disease flares, waning protection, and acute-onset syndromes have emerged as short-term adverse events after vaccination. Hence, scientific literature falls short when discussing potential long-term effects in vulnerable cohorts. The COVAD-2 survey follows on from the baseline COVAD-1 survey with the aim to collect patient-reported data on the long-term safety and tolerability of COVID-19 vaccines in immune modulation. The e-survey has been extensively pilot-tested and validated with translations into multiple languages. Anticipated results will help improve vaccination efforts and reduce the imminent risks of COVID-19 infection, especially in understudied vulnerable groups

Pain in individuals with idiopathic inflammatory myopathies, other systemic autoimmune rheumatic diseases, and without rheumatic diseases: A report from the COVAD study

Objectives To compare pain intensity among individuals with idiopathic inflammatory myopathies (IIMs), other systemic autoimmune rheumatic diseases (AIRDs), and without rheumatic disease (wAIDs). Methods Data were collected from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) study, an international cross-sectional online survey, from December 2020 to August 2021. Pain experienced in the preceding week was assessed using numeral rating scale (NRS). We performed a negative binomial regression analysis to assess pain in IIMs subtypes and whether demographics, disease activity, general health status, and physical function had an impact on pain scores. Results Of 6988 participants included, 15.1% had IIMs, 27.9% had other AIRDs, and 57.0% were wAIDs. The median pain NRS in patients with IIMs, other AIRDs, and wAIDs were 2.0 (interquartile range [IQR] = 1.0–5.0), 3.0 (IQR = 1.0–6.0), and 1.0 (IQR = 0–2.0), respectively (P < 0.001). Regression analysis adjusted for gender, age, and ethnicity revealed that overlap myositis and antisynthetase syndrome had the highest pain (NRS = 4.0, 95% CI = 3.5–4.5, and NRS = 3.6, 95% CI = 3.1–4.1, respectively). An additional association between pain and poor functional status was observed in all groups. Female gender was associated with higher pain scores in almost all scenarios. Increasing age was associated with higher pain NRS scores in some scenarios of disease activity, and Asian and Hispanic ethnicities had reduced pain scores in some functional status scenarios. Conclusion Patients with IIMs reported higher pain levels than wAIDs, but less than patients with other AIRDs. Pain is a disabling manifestation of IIMs and is associated with a poor functional status