Chronic Rheumatic Disease In Childhood Research Articles

IS SCOLIOSIS A COMMON DEFORMITY IN CHILDREN WITH JUVENILE IDIOPATHIC ARTHRITIS?

Purpose: Juvenile Idiopathic Arthritis (JIA) is the most common chronic rheumatic disease in childhood. Scoliosis can occur in children with JIA, since it mainly affects joint involvement, and contributes to the asymmetry of body and spine. This study aims to screen scoliosis in JIA, compare it with healthy controls, and evaluate the awareness among parents of children. Methods: 218 children with JIA (163 girls, 55 boys) and 144 healthy controls (124 girls, 20 boys) aged 4-16 years were involved in this study. Angle of Trunk rotation (ATR) was measured by a scoliometer by applying a forward bending test. Children with more than 5˚ ATR were referred to take X-ray. To collect demographic data from parents and assess their awareness of scoliosis, forms designed for parents were used. Results: Scoliosis was seen in 35 of the 218 (16.1%) children with JIA whose joints other than the spine were affected. 183 parents reported that they had never heard of scoliosis before the study. Scoliosis was seen in 25 children of the 183 children whose families had not heard of scoliosis before the examination. In families who had heard of scoliosis before the study, scoliosis was diagnosed in 10 of the 34 children. Conclusion: Parents of children with JIA should pay attention not only to joints and extremities but also to the spine and asymmetries in the body. However, instead of just assessing the affected joint, professionals should evaluate children with JIA comprehensively, including the spine.

Clinical, Immunological and Inflammatory Characteristics among Mexican Children with Different Subtypes of Juvenile Idiopathic Arthritis: Exploring the Correlation between Anti-Cyclic Citrullinated Peptide (anti-CCP) and Rheumatoid Factor (RF).

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood, affecting one to four of every 1000 children worldwide. It is characterized by joint inflammation lasting more than six weeks in children under 16 years. The aim of this study was to estimate the frequency of JIA subtypes in the Mexican patient population; compare clinical, immunological and inflammation markers by JIA subtype; and examine the correlation between these variables. We conducted a cross-sectional study of 50 patients with JIA (2-15 years). We estimated the frequency of each JIA subtype, assessed and compared the immunological characteristics (RF, ANA and anti-CCP) by JIA subtype at the time of diagnosis using Kruskal-Wallis or chi-square tests, and calculated Spearman correlation coefficients between the assessments. Our analysis included 50 patients, 29 (58%) girls and 21 (42%) boys, aged at the time of diagnosis 10.56 ± 3.99 years. The frequencies of JIA subtypes were RF-seropositive polyarthritis (34%), RF-seronegative polyarthritis (28%), systemic arthritis (16%), oligoarthritis (14%) and arthritis-related enthesitis (8%). We found a significant association between sex and JIA subtype (p = 0.014). There was a significant difference in anti-CCP levels by JIA subtype (p < 0.001). We also detected positive correlations between RF and anti-CCP (r = 0.63, p < 0.001) and between age and anti-CCP (r = 0.29, p = 0.041). Our study suggests that the frequency of the polyarticular subtypes of JIA is higher in Mexican children compared to other populations. Our findings highlight the importance of considering the presence of anti-CCP and RF as important criteria when deciding on treatment for JIA patients as elevated levels of these antibodies may indicate early forms of adult rheumatoid arthritis.

MRI T2 mapping and quantitative ultrasound shear wave elastography in cartilage integrity assessment for juvenile idiopathic arthritis patients

BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood that can lead to irreversible cartilage damage, with associated disability and decreased life quality. Shear wave elastography and quantitative MRI mapping can be used to identify early cartilage affection. PurposeTo evaluate diagnostic utility of distal femoral cartilage shear wave elastography and MRI T2 mapping in patients with symptomatic knee and investigate their potential clinical significance.Material and methodsTwenty patients with knee affection (study group) and 20 volunteers with the same demographic characteristics but without symptomatic knee pain (control group) were included in the study. A total of 80 knee joints of 40 individuals were evaluated. At the medial, intercondylar, and lateral condylar levels, distal femoral cartilage thickness was measured by B-mode ultrasonography and MRI, stiffness was assessed by shear wave elastography, and T2 relaxation time was measured by MRI.ResultsThe medial, intercondylar, and lateral cartilage thickness measurements were similar between the two groups and no statistically significant difference was observed while measured by US (P value 0.653,0.702,0.607) and MRI (P value 0.414, 0.4187, 0.3903). The shear wave velocity values in the study group were significantly higher than in the control group (P value 0.0202). There was a statistically significant difference between the average T2 relaxation time values for the distal femoral cartilage in the study and the control groups (P value 0.0027). SWE results were statistically significant in moderate and high disease activity while T2* revealed statistically significant p values in low as well as moderate and high disease activity; we found the best cutoff values for detection of cases using shear wave elastography velocity ratio (m/sec) about 4.445 with significant p value = 0.02, sensitivity 59.3% and specificity = 70.45%.ConclusionShear wave elastography and MRI T2 mapping are reliable, non-invasive, and acceptable methods for the assessment of pathologic cartilage. Better diagnostic information of hyaline cartilage can be obtained by adding up an extra sequence called T2 mapping to the routine MRI protocol of knee.

Identification of candidate genes and pathways associated with juvenile idiopathic arthritis by integrative transcriptome-wide association studies and mRNA expression profiles

AimJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of childhood, with genetic susceptibility and pathological processes such as autoimmunity and autoinflammation, but its pathogenesis is unclear. We conducted a transcriptome-wide association study (TWAS) using expression interpolation from a large-scale genome-wide association study (GWAS) dataset to identify genes, biological pathways, and environmental chemicals associated with JIA.MethodsWe obtained published GWAS data on JIA for TWAS and used mRNA expression profiling to validate the genes identified by TWAS. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analyses were performed. A protein–protein interaction (PPI) network was generated, and central genes were obtained using Molecular Complex Detection (MCODE). Finally, chemical gene expression datasets were obtained from the Comparative Toxicogenomics database for chemical genome enrichment analysis.ResultsTWAS identified 1481 genes associated with JIA, and 154 differentially expressed genes were identified based on mRNA expression profiles. After comparing the results of TWAS and mRNA expression profiles, we obtained eight overlapping genes. GO and KEGG enrichment analyses of the genes identified by TWAS yielded 163 pathways, and PPI network analysis as well as MCODE resolution identified a total of eight clusters. Through chemical gene set enrichment analysis, 287 environmental chemicals associated with JIA were identified.ConclusionBy integrating TWAS and mRNA expression profiles, genes, biological pathways, and environmental chemicals associated with JIA were identified. Our findings provide new insights into the pathogenesis of JIA, including candidate genetic and environmental factors contributing to its onset and progression.

Extended oligoarticular juvenile idiopathic arthritis with multiple enchondromatosis: A case report

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. Since there is no pathognomonic diagnostic criterion, the diagnosis is made by excluding other arthritis in childhood. Multiple Enchondromatosis, also known as Ollier’s disease, is an ossification disorder often seen in the metaphyseal and diaphyseal regions of long bones or the metaphyseal regions of tubular bones in the hands and feet. Pain, shortening, deformity, fracture, and transformation into malignancy may appear. Here we report a case with extended oligoarticular juvenile idiopathic arthritis (oligo JIA) who developed enchondromatosis during clinical follow-up. While the patient was in remission without medication, he presented with swelling in the hand fingers and enchondromatosis lesions with expansile lytic characteristics were seen on the radiograph. Comorbid diseases can be added in the clinical follow-up of JIA patients. Physical examination is important in terms of added comorbid disease. Our case report is important because it is the first case in which these two diseases are seen together in the literature.

Juvenil İdiyopatik Artiritte Karotis İntima Media Kalınlığı ve Preklinik Ateroskleroz için Risk Faktörleri: Tanımlayıcı Araştırma

Objective: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. Cardiovascular morbidity and mortality are becoming important health problems for children with inflammatory rheumatic diseases. The aim of this study was to evaluate carotid intima-media thickness (CIMT) in children with JIA and to examine its association with JIA subtype, markers of inflammation, and early atherosclerosis. Material and Methods: We included 112 (50 boys and 62 girls) patients who have been followed by a diagnosis of JIA for at least 6 months and 54 healthy control subjects (32 girls and 22 males) who were matched with the patients age and gender. All children underwent a carotid artery. Inflammation markers were evaluated in the patient group. Cumulative drug doses, total disease duration (TDD), active disease duration (ADD) were calculated. All CIMT values were compared in patients, subgroups, and control groups, and their relationship with inflammation markers was investigated. Results: CIMT values in found that children with JIA were significantly higher than in healthy children. There was no difference between the disease subgroups in terms of CIMT. No relationship was found between CIMT measurements and atherosclerosis risk factors, drugs, erythrocyte sedimentation rate, white blood cell and C-reactive protein. A negative correlation was found between mean platelet volume (MPV) and right CIMT. A statistically significant positive correlation was detected between the right CIMT and TDD (r=0.221, p=0.022), and ADD (r=0.248, p=0.010). Conclusion: The study showed that the patients with JIA had more risks than healthy controls for cardiovascular disease (CVD) regardless of subgroup. We concluded early and aggressive therapies may be protective for CVD. Negative correlation was found between MPV and CIMT that is consistent with recently published literature, but there is need for further studies with a larger patient population.

35 Pharmacologic management of children with juvenile idiopathic arthritis in Batna, Algeria

BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood and is usually treated with non-steroidal anti-inflammatory drugs or disease-modifying anti-rheumatic drugs. The outcome in patients with JIA has markedly improved with the advent of biologic drugs.ObjectivesThe aim of this study was to describe treatments prescribed for children with JIA in Batna, Algeria.MethodsA multicentre retrospective and descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on (JIA patient’s data collection). As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna.ResultsThe study included a total of 69 cases of JIA that were being followed in Batna health centers over the study period. Treatment modalities used for these patients included non-steroidal anti-inflammatory drugs (NSAIDs) in 54 patients (79.4%), steroids (prednisolone) in 37 patients (54.4%), conventional disease-modifying anti-rheumatic drug (c-DMARDs) in 51 patients (72.5%), biologic agents in 11 (15.9%) and intra articular injections in 17 patients (24.6%). The most frequently used c-DMARDs was methotrexate 42 (63.7%). The mean maximal dose reached was 7.5 mg (range 2.5–15). It was associated to other c-DMARDs in 2 cases. Sulfasalazine was used in 8 cases, Leflunomide in 1 case, and Hydroxychloroquine in 1 case. Biologics were used in 11 cases (15.9%).RF-positive polyarthritis 4(50%), RF-negative polyarthritis 3 (33.3), systemic arthritis 2 (33.3) were the groups that most commonly needed a biological therapy. Biologics included Rituximab (n = 2), Tocilizumab (n = 1), Anakinra (n = 1), Etanercept (n = 1) and Infliximab (n = 1). The (mean duration of the disease evolution at the initiation of the biological agent) was 4.8 ± 5.4 years (range 0.5–8 years). At the time of enrolment, 31 patients (44.9%) were in remission: 20 patients (29%) were under treatment and 11 patients (15.9%) were not, while 12 patients (17.4%) had active disease.ConclusionA high proportion of children presenting with JIA received cDMARDs. Biologics were needed in a few cases.Disclosure of InterestNone declared

37 Subtype frequencies and demographic characteristics of juvenile idiopathic arthritis in Batna, Algeria

BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. There is a disparity in the prevalence of Juvenile idiopathic arthritis (JIA) subsets between different geographical areas or ethnic groups. In Arabic and African populations, data describing JIA are scarce. However, the epidemiological studies remain the best tool to understand the disease and to improve its management.ObjectivesTo determine subtype, frequency, demographic and clinical features of JIA in Batna -Algeria- and to compare the findings with other JIA populations worldwide.MethodsA multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA status at the time of enrolment and the latest follow-up.ResultsThe study included a total of 69 cases of JIA that were being followed in Batna health centers over the study period. The female to male ratio was 1.83. The median age at diagnosis was 9 years (range 1–16). Forty-six patients (72%) were diagnosed within the first year after disease onset. At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34 oligoarthritis (49.3%), 9(13%) rheumatoid factor (RF) negative polyarticular JIA, 8(11.6%) RF positive polyarticular JIA, 6(8.7%), systemic arthritis, 6(8.7%) enthesitis-related arthritis, 3(4.3%) psoriatic arthritis and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status.ConclusionOligoarthritis was the most common JIA subtype in our study. The RF positive polyarthritis frequency was higher than in literature. Prospective multicentre studies are necessary to better identify the JIA peculiarities in our country.Disclosure of InterestNone declared

34 Subtype frequencies and demographic characteristics of juvenile idiopathic arthritis in Batna, Algeria

BackgroundJuvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. There is a disparity in the prevalence of Juvenile idiopathic arthritis (JIA) subsets between different geographical areas or ethnic groups. In Arabic and African populations, data describing JIA are scarce. However, the epidemiological studies remain the best tool to understand the disease and to improve its management.ObjectivesTo determine subtype, frequency, demographic and clinical features of JIA in Batna -Algeria- and to compare the findings with other JIA populations worldwide.MethodsA multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA status at the time of enrolment and the latest follow-up.ResultsThe study included a total of 69 cases of JIA that were being followed in Batna health centers over the study period. The female to male ratio was 1.83. The median age at diagnosis was 9 years (range 1–16). Forty-six patients (72%) were diagnosed within the first year after disease onset. At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34 oligoarthritis (49.3%), 9(13%) rheumatoid factor (RF) negative polyarticular JIA, 8(11.6%) RF positive polyarticular JIA, 6(8.7%), systemic arthritis, 6(8.7%) enthesitis-related arthritis, 3(4.3%) psoriatic arthritis and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status.ConclusionOligoarthritis was the most common JIA subtype in our study. The RF positive polyarthritis frequency was higher than in literature. Prospective multicentre studies are necessary to better identify the JIA peculiarities in our country.Disclosure of InterestNone declared

Monogenic disorders as mimics of juvenile idiopathic arthritis

BackgroundJuvenile idiopathic arthritis is the most common chronic rheumatic disease of childhood. The term JIA encompasses a heterogenous group of diseases. The variability in phenotype of patients affected by the disease means it is not uncommon for mimics of JIA to be misdiagnosed.Case presentationWe present four cases who were treated in single tertiary rheumatology centre for JIA who were subsequently diagnosed with a rare monogenic disease. All four patients shared the unifying features of presenting in early childhood and subsequently suffered with refractory disease, not amenable to usual standards of treatment. Multicentric Carpotarsal Osteolysis Syndrome and Camptodactyly-arthropathy-coxa vara-pericarditis syndrome are non-inflammatory conditions and patients typically present with arthropathy, normal inflammatory markers and atypical radiological features. Blau syndrome is an autosomal dominant condition and patients will typically have symmetrical joint involvement with a strong family history of arthritis, signifying the genetic aetiology.ConclusionsWe share our learning from these cases to add to the growing portfolio of JIA mimics and to highlight when to consider an alternative diagnosis. In cases of refractory disease and diagnostic uncertainty further imaging and genetic testing can play a crucial role in establishing the aetiology. In all of these cases the correct diagnosis was made due to careful, longitudinal clinical phenotyping and a close working relationship between rheumatology, radiology and clinical genetics; highlighting the importance of the multidisciplinary team in managing complex patients.

P033 Impact of breastfeeding on Juvenile Idiopathic Arthritis outcomes

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common, chronic rheumatic disease of childhood. The aetiology is still unclear. Over the past few years, several studies have investigated the association between JIA and environmental exposures such as infections and parental smoking. However, data concerning the influence of past breastfeeding on the presentation and the severity of JIA remain scarce and still a matter of debate (1, 2).The aim of this study was to determine the influence of breastfeeding history on the pattern and severity of JIA. Methods A cross-sectional monocentric study was carried out on JIA patients according to International League of Associations for Rheumatology (ILAR). Demographic, clinical and biological data were collected from medical records. Breastfeeding data were obtained by interviewing mothers. Patients were divided into two groups: group 1 (G1) stands for JIA patients breastfed over 6 months and group 2 (G2) stands for JIA non-breastfed patients or patients breastfed &amp;lt;6 months. Data were analyzed using the SPSS statistical package. A p-value &amp;lt; 0.05 was considered significant. Results Twenty-nine patients (10 male and 19 female) were enrolled. Mean age at disease onset was 9.1 ± 3.4 years [2–15]. Median diagnosis delay from symptoms onset was 3.5 ± 21. 1 SD months [1–72]. Median disease duration was 36 ± 79 SD months [7–336]. JIA subtypes were in decreasing order of frequency: enthesitisenthesitis-related arthritis (n = 13), oligoarticular (n = 8), Polyarticular RF- (n = 4), Polyarticular RF + (n = 2), systemic (n = 1) and psoriatic arthritis (n = 1). Inflammatory markers were raised in 48.3% (n = 14) of cases. Median ESR (raised in 41.3% of the cases) and CRP (raised in 34.4% of the cases) were 17 ± 25.1 SD mm/h [3–98] and 3 ± 18 SD mg/l [0–56]. Among patients with enthesitis-related arthritis, median BASDAI and BASFI scores were 1.8 ± 1.6 SD [0.3–6.3] and 2.5 ± 1.7 SD [0–6] respectively. Only 16 (55.2%) patients had bone densitometry. Osteoporosis and osteopenia were found in 10.3% and 13.8% of cases, respectively. Coxitis was found in 65.5% of patients (n = 19). Overall, 82.8% of children (n = 24) were breastfed. Breastfeeding was exclusive in 55.2% of cases (n = 16). Median breastfeeding duration was 12 ± 10.3 SD months [1.5–48]. Over two-thirds (n = 19) of patients were breastfed for &amp;gt;6 months. The statistical study revealed that G1 had significantly lesser occurrence of coxitis than G2 (57.9% vs 80%; P = 0.032). Age at disease onset (P = 0.26), diagnosis delay (P = 0.9), raised inflammatory markers (P = 0.76) and the frequency of osteoporosis (P = 0.87) were comparable between the two groups. No correlation was noted between breastfeeding duration and the following parameters: age at disease onset, ESR rate, CRP rate, BASDAI and BASFI. Conclusions In this study, JIA patients breastfed over 6 months had fewer coxitis occurrence. Further investigations with larger sample sizes are still required to assess the benefits of breastfeeding on JIA outcomes.

P027 Assessment of physical activity in Juvenile Idiopathic Arthritis

Abstract Background Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood, grouping together different diseases characterized by arthritis of unknown origin with onset before age of 16 years. JIA may cause functional impairment and a limited exercise capacity thus a reduced time engaged in physical activity. The aim of the study was to describe participation in physical activity among children with JIA. Methods We conducted a descriptive retrospective study including 23 patients with JIA according to the classification criteria of the International League of Associations for Rheumatology ILAR. Demographic data were collected. All patients answered a questionnaire referring to physical activity. Results The mean age of our patients was 16 years old with a sex ratio of 3. The education level was distributed as follows: 8,7% were in elementary school and 21,7% were in high school. Only 4,3% of the children were participating in high loading sports. Twenty-one per cent of the children reported spending &amp;gt;3 h per week in physical activity during leisure time. Participation in compulsory school-educational physical activity was reported in 43,5% of the children and 26,1% reported full participation. Full exemption from school sports was reported in 46.5% of cases and it was mostly associated with functional limitations. Exercise therapy was prescribed in 26.1% of patients and it was mainly swimming. The impact of physical activity on symptoms was divided into 43,5% of the children who reported an aggravation, 8,7% who reported an improvement and 34,8% who didn't report any change. Conclusion Physical activity in childhood is important to acquire knowledge and behavioral skills in order to maintain an active lifestyle. A special attention to children with JIA is required in order to promote their full potential of social interaction and improve long-term outcome.

Progressive pseudorheumatoid dysplasia misdiagnosed as juvenile idiopathic arthritis: a case report

BackgroundProgressive pseudorheumatoid dysplasia is a rare, autosomal recessively inherited, noninflammatory musculoskeletal disorder caused by mutations occurring in the WNT1-inducible signaling pathway protein 3 gene. Joint cartilage is the primary site of involvement, leading to arthralgia, joint stiffness, contractures, enlargement of the epiphyses and metaphysis of the hand joints, spinal abnormalities, short stature, early osteoarthritis, and osteoporosis. Juvenile idiopathic arthritis is the most common chronic rheumatic disease in childhood and has unknown etiology. Clinical features of progressive pseudorheumatoid dysplasia resemble those of juvenile idiopathic arthritis. Patients with progressive pseudorheumatoid dysplasia are usually misdiagnosed as having juvenile idiopathic arthritis.Case presentationA 13-year-old Yemeni female presented to the rheumatology clinic with a history of joint pains, bone pains, and bone deformity for 7 years. Weight and height were below the third percentiles. There was no tender swelling of metacarpophalangeal and interphalangeal joints, and she presented with scoliosis. Radiographs of the hands revealed the widening of the epiphyses. Progressive pseudorheumatoid dysplasia was suspected, and genetic testing for WNT1-inducible signaling pathway protein 1, 2, and 3 was requested with these findings. A homozygous, likely pathogenic variant was identified in the WNT1-inducible signaling pathway protein 3 gene, which confirmed our diagnosis.ConclusionProgressive pseudorheumatoid dysplasia is a rare form of spondyloepimetaphyseal dysplasia and is clinically misdiagnosed as juvenile idiopathic arthritis. It is crucial to consider progressive pseudorheumatoid dysplasia, especially in patients with standard inflammatory markers who are being followed up for juvenile idiopathic arthritis and not improving with antirheumatic intervention.

Explaining juvenile idiopathic arthritis to paediatric patients using illustrations and easy-to-read texts: improvement of disease knowledge and adherence to treatment

IntroductionJuvenile idiopathic arthritis (JIA) is the leading chronic rheumatic disease in childhood. To achieve adherence to therapy, in-depth understanding of disease and treatment options are important.ObjectiveDevelopment of specifically designed illustrations and standardised, easy-to-read texts for children and adolescents with JIA. Education materials were tested for comprehensibility and content validity. We hypothesised that children would be able to increase their knowledge about JIA after presentation of materials.MethodsThe illustrations were designed by a graphic artist and the informative texts consecutively transformed to easy-to-read language. The materials appear as a modular system to allow individualized information for each patient.The illustrations and texts were tested for knowledge gain and improvement of self-efficacy in children affected by JIA/ rheumatic diseases and controls. Health-related quality of life (HRQoL) was tested as an overall assessment of patients’ well-being.Results46 controls (71% female) and 38 patients (48% female) with a median age of 11 years were tested in a standardised setting. In both groups knowledge gain was significant (controls: t (44) = 11.08, p < 0.001, d = 1.65; patients: t (37) = 7.48, p < 0.001, d = 1.21). The control group had a significantly higher enhancement of disease knowledge compared to patients’ group (p = .046) The follow-up testing was only performed in one school class (20 controls) due to Covid-19 pandemic with significant improvement compared to the pre-test results (p = .002).The enhancement of self-efficacy through the teaching session was significantly higher in the patients’ group. No impairment of HRQoL was seen.ConclusionExplaining juvenile rheumatic diseases and therapeutic strategies is an important task in paediatric rheumatology. To avoid incomprehensible explanations in medical jargon, illustrations and easy-to-read texts were developed. Standardised presentation of the newly created materials resulted in a significant improvement of disease knowledge in patients and controls in addition to an enhancement of self-efficacy in patients.

Experiences of living with juvenile idiopathic arthritis: a qualitative systematic review.

The objective of this review was to investigate the available qualitative evidence to enhance understanding of the experiences of children and young adults living with juvenile idiopathic arthritis, and their carers, in any setting. Juvenile idiopathic arthritis is the most common chronic rheumatic disease in childhood. Despite the availability of effective treatments, persistent pain, growth retardation, physical disability, and psychological problems can occur. This may reduce the quality of life for patients with juvenile idiopathic arthritis by negatively affecting their family, educational, and social well-being. Patient-centered management and care for patients with juvenile idiopathic arthritis requires increasing attention to their self-reported quality of life and experiences, in addition to clinically measured disease activity. Furthermore, caring for children with juvenile idiopathic arthritis may have negative impacts on the lives of their carers and families. The experiences of carers have been poorly understood and studied. This review describes experiences and perspectives from patients and carers in order to inform the needs of families throughout their juvenile idiopathic arthritis journey. Studies describing the experiences of patients aged <21 years who have been diagnosed with juvenile idiopathic arthritis according to the International League of Associations for Rheumatology criteria, as well as the experiences of their carers, have been considered. A comprehensive search using PubMed, CINAHL, Embase, PsycINFO, Web of Science, and Google Scholar, as well as relevant conference proceedings of the American College of Rheumatology (2018-2019), the European Pediatric Rheumatology Congress 2018, the European League Against Rheumatism (2018-2019), and the Asia Pacific League of Associations for Rheumatology (2018-2019), was undertaken in December 2020 to identify pertinent published and unpublished studies. Studies published in English from 2001 to 2020 were included. The JBI approach to study selection, critical appraisal, data extraction, and data synthesis was used. Ten studies were included in this review. A total of 61 findings were extracted and aggregated to form 12 categories. From the 12 categories, five synthesized findings were developed: i) Self-management of juvenile idiopathic arthritis requires pain management, medication management, and the acquisition of knowledge and professional support; ii) A promising relationship with health care professionals but unbalanced access to services; iii) Parental financial burden and their adjustment to maintain family happiness; iv) Patients and parents support the web-based approach to communicate and develop self-management skills and acknowledge the importance of clinical trials; v) Desire to live a normal life without prejudice from school, social settings, and the workplace. This review has provided a comprehensive overview of experiences and perceptions of patients juvenile idiopathic arthritis and their parents. It is important to understand what they need to know about the disease. This review also highlights the importance of appropriate web-based programs, career counseling, infrastructures, and school facilities. Findings in this review can guide future policy and practice in order to improve care for families and children with juvenile idiopathic arthritis. Further research is required to develop management strategies for medication intolerance and to evaluate the longitudinal benefits of relevant juvenile idiopathic arthritis programs. PROSPERO CRD42019133165.

Developmental and Behavioral Problems of Preschool-Age Children with Chronic Rheumatic Diseases.

Juvenile idiopathic arthritis (JIA) is the most common autoimmune and familial Mediterranean fever (FMF) the most common autoinflammatory chronic rheumatic disease in childhood. We aimed first to identify developmental and behavioral problems of preschool-age children with common chronic rheumatic diseases, second to compare the diagnostic categories, and third to elucidate the associated factors with these difficulties. Of the 91 participants included in this study, 46 were children with rheumatic diseases (27 with JIA and 19 with FMF) and 45 were healthy children. The general developmental and emotional/behavioral problems of each child were evaluated by the Ages and Stages Questionnaire and Child Behavior Checklist-1½-5, respectively. The State-Trait Anxiety Inventory and Beck Depression Inventory were used for maternal well-being. Internalizing problem scores were increased, and the percentage of children who failed at least 1 developmental domain and experienced delays in fine motor and problem-solving domains were higher in children with chronic rheumatic diseases when compared with healthy children. Being in the group of rheumatic diseases, fewer maternal education years and higher screen time were found to be independent risk factors significantly associated with any developmental delay in the multivariable model. Maternal trait anxiety scores were positively associated with internalizing and total problems only in FMF. Preschool-age children with a diagnosis of chronic rheumatic diseases were more vulnerable for developmental and behavioral problems compared with healthy children. The management of chronic rheumatic diseases in the early years should include the screening of developmental and behavioral problems.

Spinal involvement in juvenile idiopathic arthritis: what do we miss without imaging?

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of childhood. Enthesitis-related arthritis(ERA) has been one of the most controversial subtypes of JIA with a higher risk of axial involvement. Our aim was to assess the frequency and spectrum of MRI findings of spine involvement in patients with JIA and determine if the axial involvement is always clinically symptomatic in patients with positive MRI findings. In this retrospective cross-sectional observational study we included known or suspected JIA patients who underwent spinal MRI examination between 2015 and 2017 and followed up in the Pediatric Rheumatology outpatient clinic. The demographic and clinical data were reviewed from the medical charts and electronic records. All patients were grouped as clinically symptomatic and asymptomatic for spinal involvement and MRI findings were re-evaluated for presence of inflammatory and erosive lesions. Of the 72 JIA patients, 57 (79.2%) were diagnosed with ERA, and 15 (20.8%) with non-ERA subtypes of JIA. Overall, 49 (68%) patients with JIA had positive spinal MRI findings (inflammatory and/or erosive lesions). Twenty-seven (47%) ERA patients were clinically symptomatic for spine involvement and among them, 19 (70.3%) had positive spinal MRI findings. Although 30 ERA (53%) patients were clinically asymptomatic, 23 of them (77%) had positive spinal MRI findings, as well. Eleven (73%) patients diagnosed with non-ERA JIA subtypes were clinically symptomatic for spine involvement at the time of MRI. Among them, four (36.3%) had inflammatory and/or erosive lesions on spine MRI. Four (26%) non-ERA patients were clinically asymptomatic for spine involvement, but three (75%) of them showed positive findings on spinal MRI. Inflammatory and/or erosive lesions of the thoracolumbar spine could exist in patients with JIA, regardless of the presence of symptoms. Not only because the significant proportion of ERA patients show asymptomatic axial involvement but also the presence of axial involvement in patients who were classified as non-ERA depending on current ILAR classification underlines the necessity of using MRI for accurate classification of patients with JIA.

ANTI-CYCLIC CITRULLINATED PEPTIDE ANTIBODIES VERSUS ANTI-MUTATED CITRULLINATED VIMENTIN ANTIBODIES IN JUVENILE IDIOPATHIC ARTHRITIS

Background: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood and is of unknown etiology. Anti-cyclic citrullinated peptide antibodies (Anti CCP) and anti-mutated citrullinated vimentin antibodies (Anti MCV) may help in diagnosis of JIA. Objective: Measurement of anti CCP and anti MCV level in JIA patients, and healthy persons to assess its possible role in diagnosis. Patients and methods: This study included 70 patients with JIA, and 20 healthy volunteers with matched age and sex. We assessed serum anti CCP and anti MCV level in both groups by enzyme linked immunosorbent assay (ELISA). This study carried out over a period from July, 2019 to December, 2019. Results: Serum anti-CCP and anti-MCV level showed a statistical significant difference between control and patient groups. Conclusion: Anti-CCP as well as anti-MCV antibodies may be helpful in the diagnosis of JIA and should clearly be taken into consideration.

Growth parameters of Turkish children with juvenile idiopathic arthritis

Purpose: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood which could results in growth retardation. With the present study, we aimed to investigate the growth parameters in Turkish children with JIA. Materials and Methods: Clinical and laboratory data, weight, height, and body mass index of 233 JIA patients were retrospectively collected from medical files. Growth parameters and z-scores were calculated by anthropometric references in Turkish children. The patients were diagnosed according to the International League of Associations for Rheumatology classification criteria.Results: The frequency of female patients was 59.2% (138). The mean age at diagnosis was 7.40±4.54 years, the mean age at the study time was 11.20±4.45 years. While mean initial visit weight and BMI z-scores were significantly improved at last visit, initial mean height z-score was significantly decreased. The frequency of short stature at last visit was 7.3% (number, 17). Acute phase reactants, including erythrocyte sedimentation rate, C-reactive protein levels were significantly lower at last visit than initial. Last visit growth parameters did not differ according age at diagnosis, disease duration and presence or absence of remission, relapses, corticosteroid usage, and biologic agent usage. Conclusion: Suppressing ongoing inflammation in JIA patients improves both weight and BMI z-scores of those patients, however, it may be insufficient to prevent short stature.

Serum homocysteine level in children with Juvenile Idiopathic Arthritis

Background: Juvenile Idiopathic Arthritis (JIA) is the most common chronic rheumatic disease of childhood and a leading cause of short and long-term disability. Abnormal level of serum homocysteine (Hcy) may be found in Juvenile idiopathic arthritis (JIA) patients. Some studies were done on serum homocyestine levels in children with JIA which showed conflicting results. So far, no study regarding the Hcy level in JIA patients was done in Bangladesh.&#x0D; Objectives: The study was aimed to assess the homocysteine level in children with different subtypes of JIA.&#x0D; Methods: It was a cross sectional and case control study. Fifty newly diagnosed cases of JIA attending the Pediatric Rheumatology clinic of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, from January 2016 to March 2017 were included. Fifty age and sex matched controls were selected. Serum homocysteine levels were measured in both cases and controls.&#x0D; Results: Among JIA patients, 36% had high serum homocysteine (Hcy) level whereas 100% of controls had normal level. Mean hcy level was significantly (p&lt;0.05) higher among cases than controls. Significantly higher number and level of hyperhomocystaeinemia was found among JIA patients having duration of illness more than 1 year than duration less than 1 year. Mean homocysteine level of polyarticular RF positive cases was found as 14.38±3.79 µmol/L and that of SJIA was found as 13.0±4.66 µmol/L. When compared with homocysteine levels of control group these two groups had significantly higher homocysteine level.&#x0D; Conclusion: More than one third of JIA patients had hyperhomocysteinaemia. Mean hcy level of JIA cases was significantly higher than that of controls. Significant association of hyperhomocysteinaemia was present with duration of illness and certain types of JIA.&#x0D; Bangladesh Med Res Counc Bull 2020; 46(1): 12-16