The impact of Juvenile idiopathic arthritis:Moving beyond the joint

Juveniele idiopathische artritis (JIA=jeugdreuma) is een ziekte waarbij ontstekingen van de gewrichten op de voorgrond staan. Ongeveer de helft van de patiënten groeit over de ziekte heen, bij de andere helft kent het een chronisch recidiverend beloop. Voor deze groep is het van belang dat ze hun eigen ziekte status kunnen bepalen. Wij vonden dat patiënten en hun ouders zelden artritis misten, maar ze overschatten vaak de ziekte activiteit. Vermoeidheid kwam voor in meer dan 60% van kinderen met JIA en 27% kampte met een zeer laag energie niveau op meer dan 3 dagen per week. Ons onderzoek toonde dat de vermoeidheid van patiënten met JIA niet voorspeld werd door ziekte activiteit, medicatie gebruik of uithoudingsvermogen, maar wel door verminderd fysiek functioneren zoals de patiënt die ervaart en lagere self-efficacy. Vermoeidheid had een negatieve invloed op sportfrequentie, maar leidde niet tot schoolverzuim. We vonden dat JIA op jong volwassen leeftijd geen negatieve invloed uitoefende op intieme relaties, body image en tevredenheid over de relatie status. Patiënten met JIA bewegen te weinig en hebben een slechter uithoudingsvermogen dan leeftijdsgenootjes. We hebben een 14 weken durende, interactief cognitief gedragsmatig internet programma ontwikkeld (reumaatjes@work) om kinderen aan te zetten om actiever te zijn. Dit programma had een klein positief effect op het activiteiten niveau en uithoudingsvermogen. Dit effect bleef aanwezig tot na een jaar na het beëindigen van de interventie. Patiënten die in de winter startten met reumaatjes@work verbeterden het meest. Patiënten en ouders participeerden met plezier en de therapietrouw was hoog

Case Report:Systemic Small-Vessel Vasculitis in an Adolescent With Active Ulcerative Colitis

Introduction: Small-vessel vasculitis (SVV) is a rare immunological disease that affects arterioles, capillaries and venules. It causes purpura, but can also manifest in other organs, including the gastrointestinal tract. SVV and inflammatory bowel disease (IBD) co-occur more frequently than would be expected by chance. Case description: A 16-year-old girl, who had been diagnosed with ulcerative colitis (UC) 2 years earlier at a general hospital, developed purpura, progressive abdominal pain with frequent bloody diarrhea and frontotemporal headache and swelling while on azathioprine and mesalamine maintenance therapy. Serology was positive for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) without antiprotease- or myeloperoixidase antibodies. Endoscopy revealed active left-sided UC and atypical ulcerations in the ascending colon. Biopsies of these ulcerations and of affected skin revealed leukocytoclastic vasculitis. Initially this was interpreted as an extraintestinal manifestation of UC that would subside when remission was induced, consequently infliximab was started. Over the next 3 weeks she developed severe burning pain in her right lower leg that progressed to a foot drop with numbness and the purpura progressed to bullous lesions. The diagnosis was adjusted to ANCA-associated vasculitis with involvement of skin, bowel and peripheral nerves. Infliximab was discontinued and induction treatment with high-dose prednisolone and cyclophosphamide was given until remission of SVV and UC was achieved. Subsequently, infliximab induction and maintenance was re-introduced in combination with methotrexate. Remission has been maintained successfully for over 2 years now. The foot drop only partly resolved and necessitated the use of an orthosis. Conclusion: Pediatric patients with IBD who present with purpuric skin lesions and abdominal pain should be evaluated for systemic involvement of SVV, which includes endoscopic evaluation of the gastrointestinal tract. We discuss a practical approach to the diagnosis, evaluation and management of systemic SVV with a focus on prompt recognition and early aggressive therapy to improve outcome

Pulmonary medium vessel vasculitis in an 11 year old boy: Hughes Stovin syndrome as a variant of polyarteritis nodosa?

We present the case of an 11-year-old boy presenting with haemoptysis, dyspnoea and weight loss as a manifestation of isolated pulmonary vasculitis, leading to pulmonary hypertension. He also appeared to have a longstanding dural venous sinus thrombosis. This rare presentation, especially in childhood, might represent a case of the seldomly reported Hughes-Stovin syndrome. The patient achieved remission after therapy with cyclophosphamide pulses and high-dose steroids. Based on the presented case and review of the literature, we propose that this syndrome might be a variant of polyarteritis nodosa. This report highlights diagnostic issues and describes a successful treatment regimen

Physical and psychosocial health in pediatric uveitis patients

Background: To investigate the possible associations between childhood noninfectious uveitis and cardio-respiratory fitness, physical activity, health related quality of life and fatigue. Methods: Cross-sectional analysis of 23 patients with noninfectious uveitis, aged 8-18 years. BMI, exercise capacity, muscle strength and physical activity were measured. Health-related quality of life and fatigue were assessed. The results were compared to standardized values for age matched healthy children. Results: Twenty-three patients were included. Children with uveitis had a higher bodyweight and body mass index. Children with uveitis had lower cardio-respiratory fitness and they were less physically active, but they experienced a normal quality of life and normal fatigue. Parents of children with uveitis reported a lower quality of life and more fatigue for their children than parents of healthy children. Conclusion: Our study indicates that children with noninfectious uveitis are at risk of developing lower physical and psychosocial health

Physical activity in children with Juvenile Idiopathic Arthritis compared to controls

BACKGROUND: To compare physical activity (PA) in children with juvenile idiopathic arthritis (JIA) with controls and to analyse the effect of disease specific factors on PA in children with JIA treated according to current treatment regimes. METHODS: PA was measured with a 7-day activity diary and expressed as physical activity level (PAL). Moderate to vigorous physical activity (MVPA) (hours/day) and sedentary time (hours/day) was determined. In children with JIA, medication, the number of swollen and/or painful joints, disease activity, functional ability, pain and well-being was determined. Multivariate regression analysis was performed to analyze differences in PA between JIA and controls, adjusted for influences of age, gender, season, Body Mass Index (BMI) and to analyze predictors of PA in JIA patients. RESULTS: Seventy-six children with JIA (26 boys and 50 girls, mean ± SD age 10.0 ± 1.4 years) and 131 controls (49 boys and 82 girls, mean ± SD age 10.4 ± 1.2 years) participated in this study. Children with JIA had a significantly lower PAL (0.10, p = 0.01) corrected for age, BMI, gender and season. They spent less time in MVPA (0.41 h/day, p = 0.06) and had a significantly higher mean time spent in sedentary activities (0.59 h/day, p 0.02) compared to controls. The activity level of children with JIA was related to age, gender, season, feeling of well-being and pain. CONCLUSION: Children with JIA have a lower PAL, spent less time in MVPA and spent more time on sedentary activities compared to controls despite current medical treatment and PA encouragement. TRIAL REGISTRATION: Data of the children with JIA are from the Rheumates@work study ISRCTN92733069

Role of FDG-PET/CT in children with fever of unknown origin

PURPOSE: To determine the role of 18F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET)/computed tomography (CT) in children with fever of unknown origin (FUO). METHODS: This retrospective single-center study included 110 children (0-18 years) with FUO who underwent FDG-PET/CT between 2010 and 2019. The diagnostic value of FDG-PET/CT for identifying cause of fever was calculated, treatment modifications after FDG-PET/CT were assessed, and logistic regression analyses were performed to identify clinical and biochemical factors associated with FDG-PET/CT outcome. RESULTS: In 53 out of 110 patients (48%), FDG-PET/CT identified a (true positive) cause of fever. Endocarditis (11%), systemic juvenile idiopathic arthritis (5%), and inflammatory bowel disorder (5%) were the most common causes of FUO. In 42 patients (38%), no cause of fever was found on FDG-PET/CT. In 58 out of 110 patients (53%), treatment modifications were made after FDG-PET/CT. FDG-PET/CT achieved a sensitivity of 85.5%, specificity of 79.2%, positive predictive value of 84.1%, and negative predictive value of 80.9%. On multivariate logistic regression, C-reactive protein was positively associated with finding a true positive focus of fever on FDG-PET/CT (OR = 1.01 (95% CI 1.00-1.02) per mg/L increase in CRP), while leukocyte count was negatively associated with finding a true positive focus of fever (OR = 0.91 (95% CI 0.85-0.97) per 109 leukocytes/L increase). CONCLUSION: FDG-PET/CT is a valuable diagnostic tool in the evaluation of children with FUO, since it may detect a true underlying cause in almost half (48%) of all cases where none was found otherwise. It allows full-body evaluation in patients without disease-specific symptoms on one examination. CRP and leukocyte count were significantly associated with FDG-PET/CT results, which may contribute to a priori assessment on the outcome of FDG-PET/CT. Future research could be aimed at evaluating more patient-specific factors to prospectively estimate the added value of FDG-PET/CT in children with FUO

Pharmacological conditioning for juvenile idiopathic arthritis:a potential solution to reduce methotrexate intolerance

Background: Methotrexate (MTX) therapy has proven to be a successful and safe treatment for Juvenile Idiopathic Arthritis (JIA). Despite the high efficacy rates of MTX, treatment outcomes are often complicated by burdensome gastro-intestinal side effects. Intolerance rates for MTX in children are high (approximately 50%) and thus far no conclusive effective treatment strategies to control for side effects have been found. To address this need, this article proposes an innovative research approach based on pharmacological conditioning, to reduce MTX intolerance. Presentation of the hypothesis: A collaboration between medical psychologists, pediatric rheumatologists, pharmacologists and patient groups was set up to develop an innovative research design that may be implemented to study potential improved control of side effects in JIA, by making use of the psychobiological principles of pharmacological conditioning. In pharmacological conditioning designs, learned positive associations from drug therapies (conditioning effects) are integrated in regular treatment regimens to maximize treatment outcomes. Medication regimens with immunosuppressant drugs that made use of pharmacological conditioning principles have been shown to lead to optimized therapeutic effects with reduced drug dosing, which might ultimately cause a reduction in side effects. Testing the hypothesis: This research design is tailored to serve the needs of the JIA patient group. We developed a research design in collaboration with an interdisciplinary research group consisting of patient representatives, pediatric rheumatologists, pharmacologists, and medical psychologists. Implications of the hypothesis: Based on previous experimental and clinical findings of pharmacological conditioning with immune responses, we propose that the JIA patient group is particularly suited to benefit from a pharmacological conditioning design. Moreover, findings from this study may potentially also be promising for other patient groups that endure long-lasting drug therapies