Elżbieta Smolewska

Macrophage activation syndrome (MAS) is one of the most severe complications of systemic juvenile idiopathic arthritis (sJIA). Around 10% of patients with sJIA exhibit systemic symptoms accompanied by macrophage activation syndrome (MAS), but it may occur subclinically in another 30–40%. In this article, we present a case of a 3-year-old girl diagnosed with sever MAS as an onset of sJIA complicated by disseminated intravascular coagulation (DIC). First symptoms of sJIA were observed about 5 months before setting the diagnose, and it was resembling urticaria. A comprehensive allergological diagnostics were conducted, but no cause for the skin changes was identified. A few weeks before admission to the hospital, the girl was presented with a high fever. During the hospital stay, viral, bacterial, and fungal infections were ruled out. However, the findings indicated significantly elevated markers of …

The available literature is scarce on the initial symptoms of arterial hypertension in children. Our study aimed to analyze the initial clinical profile of patients referred to the hospital with suspected hypertension and those diagnosed with hypertension for the first time during a hospitalization for other reasons. This study was a retrospective analysis of medical records in 471 patients. More than half of the patients showed no symptoms. The most common symptom reported was a headache—28% (132) of patients. The diagnosis of elevated blood pressure or hypertension was more frequent in asymptomatic patients (P = 0.001). Headaches were seen more often in healthy patients than in patients with hypertension. Newly diagnosed hypertension is mainly diagnosed in asymptomatic children. Moreover, the symptoms previously described in the literature as the most common did not prove to be predictive of hypertension …

Background: As the SARS-CoV-2 virus remains one of the main causes of severe respiratory system infections, the Food and Drug Administration strongly advises the continuation of current vaccination programs, including the distribution of updated boosters, especially in high-risk groups of patients. Therefore, there is an unceasing need for further research on the safety and, no less importantly, the clinical effectivity of the vaccines, with an extra focus on cohorts of patients with underlying health problems. This study aimed to assess the efficacy of the SARS-CoV-2 vaccine in possibly immunocompromised children with rheumatic disease while utilizing the interferon-gamma release assay (IGRA) as a marker for COVID-19 immunity in the study follow-up. Methods: This prospective study was performed in a group of 55 pediatric patients diagnosed with juvenile idiopathic arthritis. Eight participants were immunized with the Comirnaty mRNA vaccine before the research commenced, while the rest of the group (n = 47) had not been vaccinated against SARS-CoV-2. At the study baseline, the cellular response to the virus antigen was measured using a specific quantitative IGRA in whole blood; subsequently, the anti-SARS-CoV-2 test was performed, marking the antibodies’ levels in serum. Around four months after the enrollment of the last patient in the study, a follow-up survey regarding the events of COVID-19 infection within the cohort was conducted. Results: The study confirmed that all the vaccinated children developed specific T-cell (p = 0.0016) and humoral (p = 0.001 for IgA antibodies, p = 0.008 for IgG antibodies) responses to the inoculation …

Młodzieńcze idiopatyczne zapalenie stawów z zapaleniem przyczepów ścięgnistych (MIZS-ERA, enthesitis-related arthritis) oraz postać młodzieńcza łuszczycowego zapalenia stawów (MŁZS) są przewlekłymi chorobami reumatycznymi wieku dziecięcego, objawiającymi się zapaleniem stawów obwodowych lub kręgosłupa oraz objawami pozastawowymi. Brak możliwości optymalnego leczenia zapalenia stawów powoduje pogorszenie sprawności fizycznej, upośledzenie wzrostu i wpływa na obniżenie jakości życia. Młodzieńcze idiopatyczne zapalenie stawów z zapaleniem przyczepów ścięgnistych i MŁZS, łącznie stanowią około 25% ogółu pacjentów z rozpoznaniem MIZS. Objawy MŁZS mogą wystąpić już w 2. roku życia, typowe objawy pozwalające rozpoznać MIZS-ERA występują zwykle powyżej 6. roku życia, problemem powszechnie zauważalnym jest opóźnienie prawidłowego rozpoznania, czasem nawet …

Introduction The aim of this study was to assess the exposure to cardiovascular disease (CVD) risk factors in patients with juvenile idiopathic arthritis (JIA). Intima–media complex thickness (IMT), selected metabolic parameters and health behaviors were assessed in the course of the study. Methods The study included study group, which consisted of 45 patients with JIA and 37 healthy age- and sex-matched children in the control group. Analyses in both groups included anthropometric parameters, laboratory tests, IMT and a questionnaire on exposure to modifiable CVD risk factors. Results The study confirmed that CVD risk factors were present in both groups of patients. Significantly more children with JIA had abnormal BMI (p = 0.006) compared to the control group. Children in the study group were more likely to consume fruit regularly (p = 0.021) and less likely to consume fast food (p = 0.011) and sweetened beverages (p = 0.042) than children in the control group. Only 1 patient with JIA met criteria for ideal cardiovascular health. Dietary habits were not associated with IMT values, BMI, presence of joint pain or biochemical parameters in the study group. Conclusions Patients with JIA are exposed to cardiovascular risk factors equally to their healthy peers. Ideal cardiovascular health should be pursued in the pediatric population with particular attention paid to patients with chronic diseases (i.e., JIA). The application of carotid artery IMT measurement in the assessment of CVD risk requires studies on a larger group of patients.

The article presents the latest recommendations regarding the implementation of preventive vaccinations in the population of children with rheumatic diseases. This group of patients, due to the chronic disease itself with impaired immune status and the immunosuppressive treatment, is at risk of severe viral and bacterial infections, which can be prevented by vaccination. However, it should be noted that the response to these vaccinations may differ from that seen in the general population. Unfortunately, both in Poland and in the world, the vaccination status of pediatric patients with rheumatic diseases is not satisfactory. The condition for the effectiveness and safety of vaccination in this group of children is a close cooperation between the general practitioner/pediatrician and the pediatric rheumatologist in order to determine the optimal time and type of vaccinations.

Introduction The safety of COVID-19 vaccines in children with juvenile idiopathic arthritis (JIA) is the concern of patients and their parents and doctors in the current pandemic reality. The main objective of the study was to evaluate the safety of COVID-19 vaccine in patients with JIA. Method A cohort study based on short clinical follow-up of 43 children with JIA was conducted in the years 2021–2022 in two centres of paediatric rheumatology in Poland. All patients received mRNA COVID-19 vaccine. The patients' data were collected using appropriate validated questionnaire. Disease activity was evaluated using Juvenile Arthritis Disease Activity Score 27-joint count (JADAS-27). Results Ten (22.7%) children had COVID-19 infection before getting COVID-19 vaccine. After first dose of COVID-19 vaccine 25/43 (58.1%) patients presented typical adverse events: arm pain or oedema at the application side or weakness. Also, twenty five (58.1%) children had side effects after second dose of this vaccine, however the spectrum of the symptoms was wider (additionally: headache, fever, lymphadenopathy, arrhythmia). Thirteen out of 43 (30.2%) patients had active disease before and 8/43 (18.6%) after COVID-19 vaccination, while the degree of JADAS-27 activity was higher in the study group before COVID-19 vaccination (p = 0.047). Conclusions Our study found out that children and adolescents with JIA with remission without treatment or on the long-term treatment—cDMARDs or even bDMARDs, can be safely vaccinated for COVID-19. Moreover, the study found that COVID-19 vaccination does not interfere with the JIA treatment and does not …

According to the juvenile idiopathic arthritis (JIA) classification criteria there are two categories of the disease within the spondyloarthropathies spectrum—enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA). These are chronic rheumatic paediatric diseases, that manifest themselves with heterogeneous clinical symptoms and comorbidities, resulting in pain, growth and development impairment, deteriorated physical fitness and lowered health-related quality of life. Juvenile spondyloarthropathies may occur in 25 percent of patients with JIA. The age of onset usually is above 10, but it happens that correct diagnosis is delayed by a few years. Problems with a diagnosis of axial spondyloarthropathy in children may arise from the fact, that inflammation of the sacroiliac joints may be clinically silent at the beginning of the disease, which results in lesser sensitivity of the Assessment in SpondyloArthritis international Society classification criteria for axial spondyloarthropathies in paediatric practice. Concurrently it is key to rapidly introduce effective treatment, that should enable proper development and further life without active inflammation and its long-term complications. Guidelines for the pharmacotherapy of children with JIA include the use of non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticosteroids (GCSs), classic disease-modifying anti-rheumatic drugs (cDMARDs) and biological treatment. Amongst biologics, drugs that for a long time are used in various categories of JIA are medications out of the TNF alpha inhibitors group. Unfortunately, there is a large group of patients for whom such therapy is inadequate or …

Background Lipid disorders are one of the risk factors for cardiovascular diseases. The aim of the study was to estimate the lipid profile in early childhood in the population of Polish children born small for gestational age (SGA). Materials and Methods The study included 140 patients (93 SGA children and 47 controls) aged 5 to 11 years. All the subjects underwent a physical examination and blood laboratory tests for the glucose and lipid profiles. The SGA group was divided into subgroups, i.e., symmetrical and asymmetrical intrauterine growth restriction (IUGR). Results Blood sample analysis revealed higher levels of total cholesterol (SGA group 190.61 ± 24.66 mg/dL vs. controls 143.23 ± 23.90; p < 0.001). The analysis of particular cholesterol fractions showed significantly higher mean values of triglycerides and LDL cholesterol as well as lower mean values of HDL cholesterol in SGA children. Children in both groups did not differ significantly in terms of weight or body mass index. A statistically significantly higher glucose concentration was observed in SGA patients with the symmetrical type of IUGR. Analyzing the differences regarding metabolic factors, we obtained a statistically significant difference only in fasting glucose concentration (asymmetrical IUGR = 90.56 ± 10.21 vs. symmetrical IUGR = 98.95 ± 14.79; p < 0.001). Conclusions Children born SGA, even those not suffering from overweight or obesity in their early childhood, have an abnormal lipid profile, which may contribute to the development of cardiovascular diseases in adulthood.

Background Children diagnosed with juvenile idiopathic arthritis (JIA) are thought to be more likely to develop cardiovascular disease in adulthood. The factors modulating the cardiovascular risk, involving exposure to secondhand smoking, sedentary lifestyle and abnormal body mass index, might have had a stronger impact during the COVID-19 pandemic. The lack of reliable prognostic markers for a higher probability of cardiovascular events might be solved by carotid intima-media thickness (cIMT) measurement. The paramount goal of the study was to assess its usefulness in JIA patients. Materials and Methods The results of cIMT measured by a single physician in 45 children diagnosed with JIA were compared to 37 age- and sex-matched healthy counterparts. The analysis also involved anthropometric parameters, laboratory tests, and a survey regarding lifestyle-related factors. Results Four JIA patients appeared to have cIMT above the 94th percentile. A positive correlation between erythrocytes sedimentation rate (ESR) and right carotid artery percentiles was found. Passive smoking increased the cardiovascular risk regardless of JIA. Doubling the daily screen time during the pandemic led to a significant reduction in children’s physical activity. However, the number of enrolled subjects was not enough to make significant recommendations. Conclusions cIMT measurements remain an interesting perspective for future cardiovascular screening of children with JIA. It has yet to be determined whether it should be considered in all JIA patients on a reliable basis.

Młodzieńcze idiopatyczne zapalenie stawów z zapaleniem przyczepów ścięgnistych (MIZS-ERA, enthesitis-related arthritis) oraz postać młodzieńcza łuszczycowego zapalenia stawów (MŁZS) są przewlekłymi chorobami reumatycznymi wieku dziecięcego, objawiającymi się zapaleniem stawów obwodowych lub kręgosłupa oraz objawami pozastawowymi. Brak możliwości optymalnego leczenia zapalenia stawów powoduje pogorszenie sprawności fizycznej, upośledzenie wzrostu i wpływa na obniżenie jakości życia.

Diffuse fasciitis with eosinophilia (EF) is a rare condition classified as a part of the connective tissue disorders. The clinical presentation of this condition can be diverse, however the main symptoms include symmetrical swelling and hardening of distal parts of limbs accompanied by peripheral eosinophilia. The diagnostic criteria are not specified. In inconclusions cases Magnetic Resonance Imaging (MRI) and skin to muscle biopsy may be useful. The pathogenesis and ethiology remain unknown, but extensive physical exertion, certain infectious factors, such as Borrelia burgdorferi, or medications may serve as a trigger. EF affects equally women and men, mainly in their middle age, however the disease can occur at any age. The standard therapy contents gluccocorticosteroids. As a second-line treatment, methotrexate is usually chosen. In this article we compare world reports of EF in paediatric patients with the …

Background Adipose tissue is not only a storage place for fat, but also an endocrine organ, secreting bioactive molecules which influence body metabolism. Such molecules are known as adipocytokines. In the past years the coincidence between adipocytokines and fetal growth restriction disorders was found. The aim of the study was to estimate serum levels of adiponectin, leptin and resistin in children born small for gestational age, compared to children born at an appropriate size for gestational age. Methods The study consisted of 35 children aged seven to nine years, born SGA (small for gestational age) on term and 25 healthy children (14 girls, 11 boys), born with proper birthweight (AGA—appropriate for gestational age)–control group. Results Adiponectin and leptin levels were significantly higher in the SGA group compared to the AGA group (p = 0.023, p = 0.018 respectively). The resistin values were comparable in both groups of patients. There was a positive correlation between serum leptin concentration and current body weight in SGA group (r = 0.28; p = 0.108). In turn, adiponectin levels in this group of patients negatively correlated with actual body weight (r = −0.51; p = 0.002). The negative correlation between body mass index and plasma adiponectin levels was found only in children born SGA. SGA children had significantly higher values of diastolic blood pressure. There was negative correlation between serum adiponectin level and systolic blood pressure in SGA children. In the SGA group the phenomenon of catch-up growth was observed in 32 children. Conclusions Children born SGA have abnormal adipose tissue …

ResultsPatients with MTHFR rs1801133 CT/TT variant had higher values of inflammatory markers, number of joints with active arthritis, and JADAS-71 value at the baseline of MTX treatment. Children with MTRR rs1801394 AG/AA variant presented higher inflammatory marker values at JIA diagnosis.ConclusionsMTHFR rs1801133 and MTRR rs1801394 polymorphisms are associated with higher disease activity at the moment of JIA diagnosis.

ObjectiveThe decision whether or not to proceed with surgical intervention of a patient with a ruptured abdominal aortic aneurysm (rAAA) is very difficult in daily practice. The primary objective of the present study was to develop and to externally validate a new prediction model: the Dutch Aneurysm Score (DAS).MethodsWith a prospective cohort of 10 hospitals (n = 508) the DAS was developed using a multivariate logistic regression model. Two retrospective cohorts with rAAA patients from two hospitals (n = 373) were used for external validation. The primary outcome was the combined 30 day and in-hospital death rate. Discrimination (AUC), calibration plots, and the ability to identify high risk patients were compared with the more commonly used Glasgow Aneurysm Score (GAS).ResultsAfter multivariate logistic regression, four pre-operative variables were identified: age, lowest in hospital systolic blood pressure …

BackgroundJuvenile idiopathic arthritis can be refractory to some or all treatment regimens, therefore new medications are needed to treat this population. This trial assessed the efficacy and safety of baricitinib, an oral Janus kinase 1/2-selective inhibitor, versus placebo in patients with juvenile idiopathic arthritis.MethodsThis phase 3, randomised, double-blind, placebo-controlled, withdrawal, efficacy, and safety trial was conducted in 75 centres in 20 countries. We enrolled patients (aged 2 to <18 years) with polyarticular juvenile idiopathic arthritis (positive or negative for rheumatoid factor), extended oligoarticular juvenile idiopathic arthritis, enthesitis-related arthritis, or juvenile psoriatic arthritis, and an inadequate response (after ≥12 weeks of treatment) or intolerance to one or more conventional synthetic or biologic disease-modifying antirheumatic drugs (DMARDs). The trial consisted of a 2-week safety and …

BackgroundPediatric multisystem inflammatory syndrome temporally associated with COVID-19 (PIMS-TS) is a serious complication of a previous SARS-CoV-2 infection in the group of pediatric patients. Despite the fact that this disease affects only about 1 of 1000 children, it may be severe, and changes in the cardiovascular system may cause long-term complications and the need for longitudinal patient care.MethodsIt is a single-center retrospective study considering 51 patients with PIMS-TS. The aim of this study was to analyze patients with PIMS-TS, taking into account demographic data, clinical course, laboratory tests and cardiovascular system assessment (electrocardiography, echocardiography, etc.).ResultsFrom June 2020 to October 2021, 51 patients with PIMS-TS were hospitalized in our center. In the studied group, 26/51 children (51%) were girls. The mean age of patients was 7 years. Changes in …

Choroby autozapalne stanowią stosunkowo nową grupę chorób, funkcjonującą oficjalnie w medycynie od 1997 roku, kiedy to został zidentyfikowany gen odpowiedzialny za występowanie rodzinnej gorączki śródziemnomorskiej (FMF–familial Mediterranean fever). Pod koniec lat 90. XX wieku opisano dwie inne autozapalne jednostki chorobowe: zespół hiperimmunoglobulinemii D (HIDS–hyperimmunoglobulin D syndrome) oraz zespół gorączki nawrotowej zależny od receptora czynnika martwicy nowotworów (TRAPS–tumor necrosis factor [TNF] receptor-associated periodic syndrome). W ciągu ostatnich 20 lat opisano kolejne zespoły chorobowe o podłożu autozapalnym charakteryzujące się dużą heterogennością i na ogół wczesnym początkiem objawów1. Patofizjologię procesu autozapalnego cechują przewlekły przebieg oraz uszkodzenia o długoterminowym charakterze. Od kilku lat dostępne są leki skutecznie kontrolujące mechanizmy autozapalenia oraz główne objawy, co umożliwia pacjentom dotkniętym tymi genetycznie uwarunkowanymi chorobami zachowanie akceptowalnej jakości życia, chociaż wciąż nie ma skutecznej terapii większości ciężkich manifestacji. Na ogół leczenie farmakologiczne jest oparte na grupie leków biologicznych blokujących cząsteczki zaangażowane w proces zapalny, które są produkowane w nasilonej ilości w ostrej fazie epifenomenu zapalnego2.

Background There 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.Objectives The aim of this study was to evaluate the usefulness of cIMT testing as a screening method to determine cardiovascular risk …

Postępy w reumatologii dziecięcej stanowią o konieczności aktualizacji zasad postępowania i wdrażania najnowszych standardów leczenia pacjentów objętych opieką. Poniższe doniesienie przedstawia współczesne zasady postępowania terapeutycznego u pacjentów z U-MIZS, z uwzględnieniem koncepcji Treat to Target (T2T), czyli terapii zmierzającej do uzyskania remisji lub, gdy jest to niemożliwe, alternatywnym celem może być uzyskanie niskiej aktywności choroby. Autorzy pracy odnoszą się jednocześnie do możliwości realizacji tych zaleceń w warunkach polskich, z uwzględnieniem programu lekowego B. 33, podkreślając, że opcje terapeutyczne w U-MIZS powinny uwzględniać zarówno indywidualny przebieg choroby, jak i dostępne możliwości leczenia w obowiązującym w Polsce systemie prawnym i organizacyjnym. W początkowym okresie choroby zastosowanie mają niesteroidowe leki …