BackgroundPediatric Inflammatory Multisystem Syndrome Associated With SARS-CoV-2 (PIMS) is a new insidious disease which in several points may mimic Kawasaki disease. Patients diagnosed with one of the aforementioned conditions are initially treated with intravenous immunoglobulin (IVIG). However, up to 20% of children diagnosed with Kawasaki disease appear to be resistant to such therapy. Similarly, substantial portion of PIMS patients requires second line treatment including systemic glucocorticoids. There are several calculative models, including the Kobayashi Score, which are utilized to predict patients’ response to such treatment. To our best knowledge, the scoring systems derived from Kawasaki disease have not yet been assessed whether they can fulfil similar role in PIMS patients.ObjectivesThere were two essential questions to be addressed in the study: (1) Can the Kobayashi Score be utilized in making clinical decisions regarding concomitant treatment in PIMS patients? (2) Is there any modification that may increase the accuracy of the original score?MethodsFirst step of the study involved 19 patients diagnosed with PIMS between July 2020 and June 2021. The statistical analysis including each parameter of the Kobayashi Score has been performed in order to determine potential alterations of the score. Then, the numerous variants of modified score have been compared in terms of their positive and negative predictive values in order to determine new PIMS IVIG Resistance Score (PIRS). In the next phase of the study, both scores have been validated in the second cohort involving 16 patients diagnosed with PIMS between July and December 2021. The final assessment has been performed in the unified study group (35 PIMS patients).ResultsThe Kobayashi Score (see Table 1) significantly differentiated PIMS patients in terms of good response or resistance to IVIG (p=0.03967). However, the score returned a few false positive (3 out of 9) and false negative (2 out of 10) results. After step-by-step verification of clinical and laboratory parameters, authors developed a tentative PIRS (see Table 1) including the following criteria: hyponatremia, days of fever and platelet count (derived from the Kobayashi Score but with different cut-off levels) supplemented with procalcitonin level and percentage of lymphocytes. In the validatory phase of the study, both scores had equal accuracy to predict treatment response. The analysis of receiver operating characteristic curve in the unified study group has shown better performance of PIRS (Youden index 0,72) than the Kobayashi Score (Youden index 0,49).Table 1.List of criteria included in both scores assessed in the study.CriteriaThe Kobayashi ScorePIMS IVIG Resistance Score (PIRS)Sodium≤133 mmol/L2 points≤137 mmol/L1 pointDays of illness at initial treatment≤42 points≤42 pointsAspartate aminotransferase≥100 IU/L2 points--Percentage of neutrophils≥80%2 points--Percentage of lymphocytes--≤16%1 pointC-reactive protein≥100 mg/L1 point--Procalcitonin--≥3.5 ng/mL1 pointAge≤12 months1 point--Platelet count≤300 G/L1 point≤200 G/L2 pointsIncreased risk of resistance to IVIG≥4 points≥4 pointsConclusionThe Kobayashi Score is worth being considered to estimate the risk of resistance to IVIG in PIMS patients. Nonetheless, it is not free from false positive and false negative results. The postulated modified score called PIRS can become a promising alternative but it requires further validation in larger cohorts of patients.Disclosure of InterestsNone declared

BackgroundThere is growing body of evidence that adults who were diagnosed as children with juvenile idiopathic arthritis (JIA) have significantly increased risk of developing cardiovascular disease. Risk factors including prolonged sedentary screen time, insufficient physical activity and unhealthy diet are even more essential in the era of the COVID-19 pandemic. However, there is lack of simple and reliable prognostic marker identifying children at higher risk of early development of cardiovascular disease. Non-invasive tests utilized in adults to screen for early phase of atherosclerosis involve examination of the carotid intima-media thickness (cIMT). Only a few research projects have evaluated performance of cIMT measurement in JIA patients and the results remain inconclusive.ObjectivesThe aim of this study was to evaluate the usefulness of cIMT testing as a screening method to determine cardiovascular risk in JIA patients. The secondary objective was to assess the frequency of risk factors related to the patients’ lifestyle during the COVID-19 pandemic.MethodsThe study involved forty-five patients at mean age 13.4±3.2 years who were already diagnosed with JIA and thirty-seven age- and sex-matched healthy controls. Children were enrolled in the study between March 2020 and September 2021. Study database included demographic data, conventional risk factors for developing cardiovascular disease (e.g. abnormal body mass index and exposure to secondhand smoking), inflammatory markers and disease activity score. Measurements of cIMT were performed by a qualified physician according to the standardized protocol using high resolution ultrasonography.ResultsMeasurement of cIMT revealed values above 94th percentile in four children (three males and one female) who were all diagnosed with JIA. The quantity of abnormal results was not enough to verify the hypothesis of increased cardiovascular risk in JIA patients, though (p=0.06296). However, children diagnosed with JIA are more likely to have abnormal body mass index than their healthy peers (51.1% vs. 21.6%, p=0.00614). Children who doubled their sedentary screen time during the COVID-19 pandemic skipped the sufficient physical activity (p=0.03352). Correlation between elevated ESR and higher cIMT values in right carotid artery was marginally significant (r=0.292, p=0.051443). Regardless of JIA, exposure to secondhand smoking was proved as a significant risk factor of atherosclerosis (18.2% vs. 2.8%, p=0.02771).ConclusionScreening measurements of cIMT should be considered in the follow-up of JIA patients with higher disease activity with concurrent elevated ESR. Defining other indications for performing such examination requires further investigation involving larger study group. Healthy lifestyle, including reducing secondhand smoke exposure, needs to be promoted with utmost importance during the COVID-19 pandemic, especially in children with chronic diseases like JIA.References[1]Del Giudice E, Dilillo A, Tromba L et al. Aortic, carotid intima-media thickness and flow- mediated dilation as markers of early atherosclerosis in a cohort of pediatric patients with rheumatic diseases. Clin Rheumatol. 2018 Jun;37(6):1675-1682. doi: 10.1007/s10067-017-3705-7.[2]Hussain KS, Gulati R, Satheesh S et al. Early-onset subclinical cardiovascular damage assessed by non-invasive methods in children with Juvenile Idiopathic Arthritis: analytical cross-sectional study. Rheumatol Int. 2021 Feb;41(2):423-429. doi: 10.1007/s00296-020-04689-z.Disclosure of InterestsNone declared

BackgroundWhile serological assays detecting antibodies in blood serum remain the standard exam for evaluating immunity against SARS-CoV-2 infection, the latest publications reveal certain limitations to this broadly used method. Recent studies showing the decline of antibodies titers in time, together with scientific reports which detect SARS-CoV-2-specific T-cells in seronegative individuals, evoke questions with regard to the reliability of the sole assessment of the humoral response to SARS-CoV-2. A novel T-cell-based Interferon-γ Release Assay (IGRA) is proposed to adapt our approach to the individual immunity to the new coronavirus.ObjectivesThe objectives of this study were to assess T-cells and antibodies responses to the SARS-CoV-2 virus among pediatric patients at different stages of juvenile idiopathic arthritis (JIA) undergoing diverse treatment regimes. In addition, the authors aimed to evaluate humoral and cellular responses to the Covid-19 mRNA vaccine in the cohort of immunocompromised patients.MethodsThis prospective study included 30 pediatric patients between the ages 2-16 at different stages of JIA. The cohort group consisted of vaccinated and unvaccinated individuals and children with both positive and negative history of past Covid-19 infection. SARS-CoV-2 T-cell response was measured using a specific quantitative IGRA in whole blood, followed by anti-SARS-CoV-2 ELISA test measuring the presence and quantity of IgG, IgM and IgA antibodies in serum.ResultsThe magnitude of the SARS-CoV-2 specific T-cell response measured by the IGRA test correlated significantly with the levels of IgG (p<0.00005, R=0.653) and IgA antibodies (p<0.00001, R=0.675) in serum, while no correlation with IgM antibodies level in serum was found. All vaccinated patients developed a cellular response to the vaccine (p=0.0108), regardless of the number of administered doses. The T-cells response in children undergoing biological treatment (specifically: etanercept, adalimumab or tocilizumab) was recognizably weaker compared to the group not receiving such treatment. Nevertheless, the difference did not reach statistical significance (p=0.0715). Similar dependencies were found in children treated with hydroxychloroquine (n=6), however, due to the small study group, a statistical significance could not have been reached.ConclusionSuppression of cellular response observed in different drug protocols may prove to become a protective factor against SARS-CoV-2 infection. Additionally, the authors postulate that the strong correlation found between T-cell response and IgA antibodies titer can be related to SARS-CoV-2 strong affinity with mucosal membranes. Thus, the sole testing of IgG and IgM antibodies levels in serum with regard to Covid-19 may not be the most precise diagnostic method. The presented study group needs to be expanded with more patients, mainly children receiving biological agents or hydroxychloroquine to validate the demonstrated preliminary results.References[1]Prendecki M, Clarke C, Edwards H et al. Humoral and T-cell responses to SARS-CoV-2 vaccination in patients receiving immunosuppression. Ann Rheum Dis. 2021 Oct;80(10):1322-1329. doi: 10.1136/annrheumdis-2021-220626.Disclosure of InterestsNone declared