Juvenile Rheumatic Diseases Research Articles

The impact of juvenile onset rheumatic and musculoskeletal diseases on education, vocation and employment

The impact of juvenile onset rheumatic and musculoskeletal diseases on education, vocation and employment Professor Suzanne Verstappen discusses the impact and challenges associated with juvenile-onset rheumatic and musculoskeletal diseases when navigating education and employment. Juvenile onset rheumatic and musculoskeletal diseases (jRMDs), such as juvenile idiopathic arthritis (JIA), lupus and vasculitis, are chronic diseases with onset before the age of 16 years. These conditions can cause persistent joint pain, swelling of the joints and stiffness. Other symptoms include fatigue and functional disability. These symptoms may fluctuate, and sometimes, children and young adolescents with jRMDs have severe symptoms, whilst, on different days, they may feel better. Some children may experience the symptoms only for a couple of months or years, whilst others have symptoms for many years and continue to have symptoms during adulthood. Some types of jRMDs can cause serious complications, such as joint damage, growth problems and inflammation of the eyes. Although disease management has improved in the last decade with more effective drugs and multidisciplinary care, the symptoms, adverse events due to medication use and clinical visits may impact school attendance, career choices and future employment.

JAK inhibitors in refractory juvenile rheumatic diseases: Efficacy, tolerance and type-I interferon profiling, a single center retrospective study

Objectives– Janus Kinase inhibitors (JAKi) are a new class of drugs available for pediatric rheumatic diseases.This study aimed to describe the safety and effectiveness of JAKi in these diseases, with a focus on longitudinal interferon-stimulated genes (ISG) assessment. Methods– We present a single-center retrospective study of children with refractory pediatric rheumatic diseases including connective tissue diseases, monogenic type I interferonopathies or juvenile idiopathic arthritis, receiving JAKi. According to physicians’ assessment, treatment effectiveness was classified at 12 months as a complete response in the total absence of disease activity, partial response in case of significant (>50%) but incomplete improvement or no response in the case of non-response or improvement of less than 50% of the clinical and biological parameters. ISG were monitored longitudinally using Nanostring technology. Results- 22 children were retrospectively included in this study, treated either by baricitinib or ruxolitinib. Complete response was achieved at 12 months in 9/22 (41%) patients. 6/22 (27%) patients were non-responders and treatment had been discontinued in five of them. Within the interferon (IFN)-related diseases group, ISG-score was significantly reduced 12 months after JAKi onset (p = 0.0068). At 12 months, daily glucocorticoid doses had been reduced with a median dose of 0.16 mg/kg/day (IQR 0.11; 0.33) (p = 0.0425). 7/22 (32%) patients had experienced side effects, infections being the most common. Increase of the body mass index was also recorded in children in the first 6 months of treatment. Conclusion– JAKi represent a promising treatment of immune-mediated pediatric diseases, enabling to decrease type-I IFN transcriptomic signature in responding patients, especially in the context of juvenile dermatomyositis. JAKi represent steroid-sparing drugs but they induce metabolic changes linked to weight gain, posing a concern in the treatment of young patients and teenagers. More data are required to define the efficacy and safety of JAKi in the management of refractory pediatric rheumatic diseases.

Single center clinical analysis of macrophage activation syndrome complicating juvenile rheumatic diseases

BackgroundMacrophage activation syndrome (MAS), an example of secondary hemophagocytic lymphohistiocytosis, is a potentially fatal complication of rheumatic diseases. We aimed to study the clinical and laboratory characteristics, treatment schemes, and outcomes of different rheumatic disorders associated with MAS in children. Early warning indicators of MAS have also been investigated to enable clinicians to make a prompt and accurate diagnosis.MethodsFifty-five patients with rheumatic diseases complicated by MAS were enrolled between January 2017 and December 2022. Clinical and laboratory data were collected before disease onset, at diagnosis, and after treatment with MAS, and data were compared between patients with systemic juvenile idiopathic arthritis (sJIA), Kawasaki disease (KD), and systemic lupus erythematosus (SLE). A random forest model was established to show the importance score of each variable with a significant difference.ResultsMost (81.8%) instances of MAS occurred during the initial diagnosis of the underlying disease. Compared to the active stage of sJIA, the platelet count, erythrocyte sedimentation rate, and fibrinogen level in sJIA-MAS were significantly decreased, whereas ferritin, ferritin/erythrocyte sedimentation rate, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and D-dimer levels were significantly increased. Ferritin level, ferritin/erythrocyte sedimentation rate, and platelet count had the greatest predictive value for sJIA-MAS. The level of IL-18 in the sJIA-MAS group was significantly higher than in the active sJIA group, whereas IL-6 levels were significantly lower. Most patients with MAS were treated with methylprednisolone pulse combined with cyclosporine, and no deaths occurred.ConclusionsThrombocytopenia, ferritin levels, the ferritin/erythrocyte sedimentation rate, and elevated aspartate aminotransferase levels can predict the occurrence of MAS in patients with sJIA. Additionally, our analysis indicates that IL-18 plays an important role in the pathogenesis of MAS in sJIA-MAS.

Risk perception, well-being, depression and anxiety in children and adolescents with rheumatic diseases during the COVID-19 pandemic - results from the prospective multicenter KICK-COVID study in Germany

ObjectiveTo investigate the psychosocial burden in children and adolescents with juvenile rheumatic diseases during the COVID-19 pandemic.MethodsAs part of the multicentre observational KICK-COVID study linked to the National Pediatric Rheumatology Database, adolescents < 21 years and parents of children < 12 years with rheumatic diseases answered questions on perceptions of health risk (PHR) due to SARS-CoV2, stress, well-being (WHO-5) and symptoms of depression (PHQ-9) and anxiety (GAD-7). Data were collected at routine visits from June to December 2021 and assessed for association with demographic and clinical parameters, treatment and patient-reported outcomes by multivariable regression analyses.ResultsData from 1356 individuals (69% female, 50% adolescents) were included. Median PHR on a numeric rating scale (NRS, 0–10) was 4 (IQR 2–6), median perceived stress was 3 (IQR 1–6). Adolescents reported a worse well-being with a significantly lower median WHO-5-score (60, IQR 40–76) than parents reported for their children < 12 years (80, IQR 68–84). Moderate to severe symptoms of depression and anxiety were reported by 14.3% and 12.3% of the adolescents, respectively. PHR was significantly higher in patients with systemic lupus erythematosus, methotrexate or biologic disease-modifying anti-rheumatic drug therapy than in patients without these characteristics, whereas lower WHO-5 or higher PHQ-9 or GAD-7 scores were only associated with poorer patient-reported health status and physical functioning.ConclusionThe perception of health risk due to SARS-CoV2 infection was not paralleled by an impairment of mental health, which were, however, significantly correlated with self-rated health status and functional capacity, highlighting the importance of patient-reported outcome assessment.Trial registrationGerman Clinical Trials Register (DRKS), no. DRKS00027974. Registered on 27th of January 2022.

Canakinumab treatment real world evidence in 3 monogenic periodic fever syndromes in 2009–2022: an interim analysis using the French JIR cohort database

BackgroundOur study aimed to provide real-world evidence on the treatment patterns, effectiveness and safety of canakinumab in France in Familial Mediterranean Fever (FMF), Mevalonate Kinase Deficiency (MKD), and Tumor necrosis factor Receptor Associated Periodic Syndrome (TRAPS).MethodsThis study used the JIR cohort, a multicentre international registry created in 2013 to collect data on patients with juvenile inflammatory rheumatic diseases. French patients diagnosed with FMF, MKD or TRAPS and treated with canakinumab were included in this study.Results31 FMF, 26 MKD and 7 TRAPS patients received canakinumab during the study period. Most of them initiated canakinumab at the recommended dose of 2 mg/kg or 150 mg, but less than half of FMF and MKD patients initiated it at the recommended frequency (every 4 weeks). Two years after initiation, the rate of patients still on treatment was 78.1% in FMF, 73.7% in MKD, and 85.7% in TRAPS patients. While the dose per injection remained globally the same over the course of the treatment, some adjustments of the dose intervals were observed. Six patients had a severe adverse event reported. Of those, three were possibly related to canakinumab.ConclusionThis interim analysis showed a good maintenance of canakinumab treatment 2 years after initiation and confirmed its safety profile in real-life practice in France in patients diagnosed with FMF, MKD and TRAPS. The high variety of dose and interval combinations observed in canakinumab treated patients let suppose that physicians adapt the posology to individual situations rather than a fixed treatment plan.

Consensus evidence-based recommendations for transition of care for adolescents with juvenile idiopathic arthritis: meeting patients’, parents’, and rheumatologists’ perspectives

BackgroundTransition of care means the process of educating and empowering adolescents and young adults to take an active role in their own healthcare, develop decision-making skills, and eventually transition from paediatric to adult healthcare providers. Most people do not switch doctors until they are young adults, but it can be beneficial to start preparing children earlier. We aimed to develop a specific toolkit tailored to paediatric and adult rheumatologists to assist them in transitioning of care of young people with juvenile onset rheumatic musculoskeletal diseases from the paediatric to adult rheumatology care.ResultsThe expert panel was confined to an online survey (n = 18), all the experts completed the two rounds. At the conclusion of round 2, a total of 10 points were gathered. The range of respondents (ranks 7–9) who agreed with the recommendations was 88.9 to 100%. All 10 clinical standards identified by the scientific committee were written in the same way. Based on the answers to the structured key questions and the literature review, a structured template was developed presenting transition of care integrated pathway.ConclusionThe developed rheumatology-specific guideline offers adolescents and young adults a focussed, multidisciplinary transition of care approach with equity of access, quality of care and flexibility and set up standards for transitional care for young adults with juvenile rheumatological diseases.

Can vitamin D be an adjuvant therapy for juvenile rheumatic diseases?

Vitamin D, known for its essential role in calcium and bone homeostasis, has multiple effects beyond the skeleton, including regulation of immunity and modulation of autoimmune processes. Several reports have shown suboptimal serum 25 hydroxyvitamin D [25(OH)D] levels in people with different inflammatory and autoimmune rheumatic conditions, and an association between 25(OH)D levels, disease activity and outcomes. Although most available data pertain to adults, insights often are extended to children. Juvenile rheumatic diseases (JRDs) are a significant health problem during growth because of their complex pathogenesis, chronic nature, multisystemic involvement, and long-term consequences. So far, there is no definitive or clear evidence to confirm the preventive or therapeutic effect of vitamin D supplementation in JRDs, because results from randomized controlled trials (RCTs) have produced inconsistent outcomes. This review aims to explore and discuss the potential role of vitamin D in treating selected JRDs. Medline/PubMed, EMBASE, and Scopus were comprehensively searched in June 2023 for any study on vitamin D supplementary role in treating the most common JRDs. We used the following keywords: "vitamin D" combined with the terms "juvenile idiopathic arthritis", "juvenile systemic scleroderma", "juvenile systemic lupus erythematosus", "juvenile inflammatory myopathies", "Behcet disease", "periodic fever syndromes" and "juvenile rheumatic diseases". Observational studies have found that serum 25(OH)D concentrations are lower in juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile systemic scleroderma, Behcet disease and proinflammatory cytokine concentrations are higher. This suggests that vitamin D supplementation might be beneficial, however, current data are insufficient to confirm definitively the complementary role of vitamin D in the treatment of JRDs. Considering the high prevalence of vitamin D deficiency worldwide, children and adolescents should be encouraged to supplement vitamin D according to current recommendations. More interventional studies, especially well-designed RCTs, assessing the dose-response effect and adjuvant effect in specific diseases, are needed to determine the potential significance of vitamin D in JRDs treatment.

E07 Development of a coordinated transitional care program on adolescents with juvenile idiopathic arthritis: looking through the patient lens

Abstract Background Transition of care means the process of educating and empowering adolescents and young adults to take an active role in their own healthcare, develop decision-making skills, and eventually transition from paediatric to adult healthcare providers. Most people don't switch doctors until they are young adults, but it can be beneficial to start preparing children earlier. Objectives To provide a toolset specifically suited for adult and paediatric rheumatologists to help them transition the care of children with rheumatic diseases from adult to paediatric rheumatology care. Methods The preliminary scientific committee identified a total of ten major clinical questions in this study, which was conducted to obtain expert consensus. To reach consensus on the transition of care for young adults and adolescents with rheumatic diseases, the core leadership team sought out researchers and clinicians with expertise in both paediatric and adult rheumatic diseases management. Results The expert panel's participation was restricted to an online survey (n = 18); each expert completed both rounds. After round 2, a total of 10 points had been collected. 88.9% to100% of respondents (ranks 7–9) indicated they agreed with the recommendations. The scientific committee's 10 clinical standards were all prepared in the same style. A structured template describing the integrated pathway for transition of care was developed using the responses to the structured key questions and the results of the literature review. Conclusion The created rheumatology-specific guideline provides young adults with juvenile rheumatic diseases with a focused, multidisciplinary transition of care strategy with equity of access, quality of care, and flexibility and establishes standards for transitional care. Ethics This study was performed in accordance with the Helsinki Declaration. The ‘Clinical, Evidence-based, Guidelines’ (CEG) initiative protocol was approved the local ethical committee: ethical approval code: 34842/8/21, ethical board Tanta University.

Childhood rheumatic diseases: bites not only the joint, but also the heart.

Cardiovascular involvement in juvenile rheumatic diseases is the primary manifestation in paediatric vasculitis and a major organ manifestation in paediatric connective tissue diseases. Though coronary vasculitis is the prototypical manifestation of Kawasaki disease, it can also be seen in patients with polyarteritis nodosa. Pericarditis is the most common manifestation seen in juvenile rheumatic diseases like systemic onset JIA, and lupus. Cardiac tamponade, valvular insufficiency, aortic root dilatation and arrhythmias are seen rarely. Cardiac involvement is often recognized late in children. The development of cardiac disease in juvenile systemic sclerosis is associated with a poor outcome. In long term, childhood onset of rheumatic diseases predisposes to diastolic dysfunction and premature atherosclerosis during adulthood. Key Points • Pericarditis is the most common cardiac manifestation in SLE and can lead to tamponade. • Conduction defects are common in juvenile mixed connective tissue disease and systemic sclerosis. • Pulmonary hypertension is a significant contributor to mortality in juvenile systemic sclerosis. • In Kawasaki disease, early treatment can reduce risk of coronary artery aneurysms.

Do Patterns of Early Disease Severity Predict Grade 12 Academic Achievement in Youths With Childhood-Onset Chronic Rheumatic Diseases?

To test the association of early disease severity with grade 12 standards test performance in individuals with childhood-onset chronic rheumatic diseases (ChildCRDs), including juvenile arthritis and systemic autoimmune rheumatic diseases. We used linked provincial administrative data to identify patients with ChildCRDs born between 1979 and 1998 in Manitoba, Canada. Primary outcomes were Language and Arts Achievement Index (LAI) scores and Math Achievement Index (MAI) scores from grade 12 standards test results as well as enrollment data. The secondary outcome was enrollment in grade 12 by 17 years of age. Latent class trajectory analysis identified disease severity groups using physician visits following diagnosis. Multivariable linear regression tested the association of disease severity groups with LAI and MAI scores, and logistic regression tested the association of disease severity with age-appropriate enrollment, after adjusting for sociodemographic factors and psychiatric morbidities. The study cohort included 541 patients, 70.1% of whom were female. A 3-class trajectory model provided the best fit; it classified 9.7% of patients as having severe disease, 54.5% as having moderate disease, and 35.8% as having mild disease. After covariate adjustment, severe disease was associated with poorer LAI and MAI scores but not with age-appropriate enrollment. Among patients with ChildCRDs, those with severe disease performed more poorly on grade 12 standards tests, independent of sociodemographic and psychiatric risk factors. Clinicians should work with educators and policy makers to advocate for supports to improve educational outcomes of patients with ChildCRDs.

Update of evidence- and consensus-based guidelines for the treatment of juvenile idiopathic arthritis (JIA) by the German Society of Pediatric and Juvenile Rheumatic Diseases (GKJR): New perspectives on interdisciplinary care

BackgroundNew therapeutic strategies for juvenile idiopathic arthritis (JIA) have evolved within the past ten years, and as a result, an update of the 2011 recommendations of the German management guidelines was initiated. MethodsA systemic literature review was performed, overarching principles were proposed and pre-selected via an online survey followed by two multidisciplinary consensus conferences. Pharmacological and non-pharmacological treatments were discussed, statements were proposed and ultimately agreed upon by nominal group technique (NGT). Results12 overarching therapeutic principles, as well as 9 recommendations on pharmacological and 5 on non-pharmacological treatments for JIA were agreed upon. ConclusionThis report summarizes the recent update of the interdisciplinary, consensus-based German guidelines on the management of JIA. The multi- and interdisciplinary participation of all caregivers was central for this patient-focused update. With these guidelines, physicians can choose an evidence-based approach, which allows better tailored treatment in this vulnerable cohort of children and adolescents.

P59 Growing up in rheumatology: An Evaluation of a Young Adult Rheumatology Service

P59 Growing up in rheumatology: An Evaluation of a Young Adult Rheumatology Service

Standardized nailfold capillaroscopy in children with rheumatic diseases: a worldwide study.

To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively. This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD.

Yellow fever vaccination in Brazil: Short-term safety and immunogenicity in juvenile autoimmune rheumatic diseases

Yellow fever vaccine (YFV) is a live attenuated vaccine usually contraindicated for juvenile autoimmune rheumatic disease (JARD) patients. During the recent epidemic in Sao Paulo-Brazil, YFV was indicated for patients under low immunosuppression. Thirty JARD patients with inactive diseases undergoing low immunosuppression and 30 healthy controls (HC) were vaccinated with a fractional dose 17DD YFV (∼5495 IU) and evaluated 30 days later. JARD patients and controls had comparable median age (12.4 vs. 12 years, p = 0.250). Disease parameters remained stable 30 days after 17DD YFV (p > 0.05) and only mild adverse events were reported in both groups (p > 0.05). JARD and HC had similar seroprotection [93% vs. 100%;p = 0.49], seroconversion rates [96% vs. 100%;p = 0.489], and GMT [1249 vs.1293;p = 0.821]. Both groups had similar white-blood-cells kinetics with transient decreases in lymphocytes at D5 and neutrophils at D10, followed by full recovery at D30 (P < 0.05). In conclusion, 17DD YFV was safe and immunogenic in JARD. This study may contribute to recommendations for patients living/travelling to endemic areas.

Psychosocial burden of inflammatory bowel disease in adolescents and young adults.

This study examined the psychosocial burden of inflammatory bowel disease (IBD) in young people aged 15-25 years attending a tertiary specialist health centre for adolescents and young adults in Brisbane. To describe the impact of IBD on psychosocial well-being in young people and to compare well-being in the IBD cohort to well-being among young people with other chronic conditions, with a view to identifying characteristics and challenges unique to those with IBD. Young people with IBD provided demographic information and psychosocial data through a cross-sectional self-report survey. Psychosocial data included the Kessler Psychological Distress Scale, Perceived Stress Scale, Brief Illness Perception Questionnaire, World Health Organisation Well-being Index, Paediatric Quality of Life Inventory, Short Quality of Life Questionnaire for IBD, Multidimensional Scale of Perceived Social Support, Connor Davidson Resilience Scale 2 and the Multidimensional Health Locus of Control Scale. Surveys were collected from 51 young people with IBD and compared with surveys from 210 young people with juvenile rheumatic disease (n= 31), phenylketonuria (n=21), cystic fibrosis (n=33), renal transplants (n=14) and craniomaxillofacial conditions (n=111). On the psychosocial domains, 41% of young people with IBD had poor well-being and 37% were at risk of depression. When assessed against the comparison group, young people with IBD reported higher depressive symptoms (P=0.04), worse illness perceptions (P < 0.01) and lower internal locus of control (P < 0.01). Early recognition and treatment of depression and other psychosocial comorbidities within integrated pathways of care is crucial in adolescents and young adults with IBD and likely to improve the course of IBD and their overall health and well-being. Interventions aimed at enhancing self-efficacy and increasing public awareness are also likely to be helpful.

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.

A Population-based Study of Grade 12 Academic Performance in Adolescents With Childhood-onset Chronic Rheumatic Diseases.

The aims of this study were (1) to compare grade 12 standardized test results of patients diagnosed with childhood-onset chronic rheumatic diseases (ChildCRD) and unaffected peers; and (2) to identify factors associated with test results of patients with ChildCRD and unaffected peers. This was a population-based retrospective cohort study. All patients with ChildCRD (juvenile arthritis and systemic autoimmune rheumatic diseases) from the only pediatric rheumatology center in Manitoba for birth cohorts January 1979 to December 1998 were linked to the provincial administrative databases containing records of healthcare use and education outcomes. Patients were matched by age, sex, and postal codes to their peers who did not have ChildCRD. The primary outcomes were the grade 12 Language Arts Achievement Index (LAI) and the Math Achievement Index (MAI) scores. ChildCRD, sociodemographic, and mental health factors were tested for their associations with LAI and MAI scores using multivariable linear regression. Five hundred and forty-one patients with ChildCRD were matched to 2713 unaffected peers. Patients with ChildCRD had lower LAI and MAI scores compared to their peers. More patients with ChildCRD failed or did not take the language arts (51% vs 41%, P < 0.001) and math (61% vs 55%, P = 0.02) tests. On multivariable analysis, ChildCRD, lower socioeconomic status, younger maternal age at first childbirth, family income assistance, involvement with child welfare services, and mental health morbidities (between ChildCRD diagnosis and standardized testing), were associated with worse LAI and MAI results. This population-based study showed that patients with ChildCRD performed less well than their peers on grade 12 standardized testing, independent of sociodemographic and mental health comorbidities.

Exploring the Evidence for an Immunomodulatory Role of Vitamin D in Juvenile and Adult Rheumatic Disease.

Vitamin D is synthesized in the skin following exposure to UVB radiation or is directly absorbed from the diet. Following hydroxylation in the liver and kidneys, vitamin D becomes its bioactive form, 1,25(OH)2D, which has been described to have potent immunomodulatory capacity. This review will focus on the effect of vitamin D in modulating the dysregulated immune system of autoimmune rheumatic diseases (ARD) patients across age, in particular in arthritis (rheumatoid arthritis and juvenile idiopathic arthritis), and systemic lupus erythematosus (with adult and juvenile onset). As well as delineating the impact of vitamin D on the innate and adaptive immune functions associated with each disease pathology, this review will also summarize and evaluate studies that link vitamin D status with disease prevalence, and supplementation studies that examine the potential benefits of vitamin D on disease outcomes. Exploring this evidence reveals that better designed randomized controlled studies are required to clarify the impact of vitamin D supplementation on ARD outcomes and general health. Considering the accessibility and affordability of vitamin D as a therapeutic option, there is a major unmet need for evidence-based treatment recommendations for the use of vitamin D in this patient population.

Patients with psychiatric diagnoses have lower quality of life than other patients with juvenile rheumatic disease: a prospective study.

ObjectivesTransition of adolescents with chronic diseases from paediatric healthcare to adult care requires attention to maintain optimal treatment results. We examined changes in health-related quality of life (HRQoL) and disease activity among JIA patients with or without concomitant psychiatric diagnoses after transfer to an adult clinic.MethodsWe prospectively followed 106 consecutive patients who were transferred from the New Children’s Hospital to the Helsinki University Hospital Rheumatology outpatient clinic between April 2015 and August 2019 and who had at least one follow-up visit. HRQoL was measured using 15D, a generic instrument.ResultsThe patients’ median age at transfer was 16 years and disease duration 4.0 years. Patients were followed for a median of 1.8 years. Disease activity and overall HRQoL remained stable, but distress (dimension 13 of 15D) increased during follow up (P=0.03). At baseline, patients with at least one psychiatric diagnosis had lower overall 15D scores [mean 0.89 (s.d. 0.14) vs 0.95 (s.d. 0.05), P <0.01] and higher disease activity [DAS28mean 1.88 (s.d. 0.66) vs 1.61 (s.d. 0.31), P = 0.01] than patients without psychiatric diagnoses. The difference in overall 15D persisted over the study period. ConclusionTransition-phase JIA patients with psychiatric diagnoses had lower HRQoL than other JIA patients. Despite reduced disease activity and pain, HRQoL of patients with psychiatric diagnoses remained suboptimal at the end of follow-up. Our results highlight the necessity of comprehensive care and support for transition-phase JIA patients.

Characteristics and treatment practices among patients with juvenile rheumatic heart Disease enrolled in a tertiary care hospital-based registry

Characteristics and treatment practices among patients with juvenile rheumatic heart Disease enrolled in a tertiary care hospital-based registry