The effects of inspiratory muscle training on cardiorespiratory functions in juvenile idiopathic arthritis: A randomized controlled trial.

INTRODUCTION: Although inspiratory muscle training (IMT) has proven effective in adult rheumatic diseases, its impact on juvenile idiopathic arthritis (JIA) remains unexplored. The present study aimed to investigate the effects of IMT in children with JIA. METHODS: Thirty-three children (13-18 years) with JIA were divided into two groups as exercise (n = 17) and control (n = 16). The exercise group performed IMT at home daily for 8 weeks. The initial IMT load was set as 60% of maximal inspiratory pressure (PImax ) and increased by %10 of the initial load every 2 weeks. The control group received no additional intervention. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1 ), FVC/FEV1 , PImax , and maximal expiratory pressure (PEmax ) were evaluated. Peak oxygen consumption (VO2max ), metabolic equivalents (METs), and maximal heart rate were measured with cardiopulmonary exercise test. Functional capacity and quality of life were assessed with 6-min walk distance and Pediatric Quality of Life Inventory 3.0 Arthritis Module. All participants were evaluated at baseline and post-treatment. RESULTS: FVC ( ↑ 0.20 (95% CI: 0.07/0.32) liters), FEV1 ( ↑ 0.14 (95% CI: 0.02/0.25) liters), PImax (↑19.11 (95% CI: 9.52/28.71) cmH2 O), PEmax (↑12.41 (95% CI: 3.09/21.72) cmH2 O), VO2peak (↑158.29 (95% CI: 63.85/252.73) ml/min), and METs (↑0.92 (95% CI: 0.34/1.49) [ml/kg/min]) significantly improved only in the exercise group (p < .05). The difference over time in FVC, FEV1 , PImax , VO2peak , and METs were significantly higher in exercise group compared to control group (p < .05). CONCLUSIONS: IMT seems to be an effective option for improving respiratory functions and aerobic exercise capacity in JIA.

Evaluation of clinical outcomes in systemic juvenile idiopathic arthritis patients treated with biological agents in Turkey: the TURSIS study.

OBJECTIVES: Biological drugs are one of the most effective treatment methods for systemic juvenile idiopathic arthritis (SJIA) and can significantly prevent morbidity and mortality. This study aimed to evaluate the efficacy and safety of biologics in patients with SJIA and provide real-life data that might help improve the outcomes. METHODS: TURSIS was a retrospective multicentre study carried out in patients with SJIA for whom a biological treatment had been initiated between 1st March 2013 and 30th December 2018. Data include patients' characteristics, laboratory-clinical results, outcomes, and safety-related variables. The 24-month follow-up data of the patients and the efficacy and safety of biological drugs were evaluated. RESULTS: 147 patients were enrolled. The clinical course of the disease was as follows; it was monocyclic in 38.1%, polycyclic in 49%, and persistent in 12.9% of patients. First-choice biologics were interleukin (IL)-1 blockers in the majority of patients (56.5%), followed by the anti-IL-6 (25.2%) and anti-TNF-alpha drugs (18.4%). Anakinra was the most preferred biologic agent in patients with macrophage activation syndrome (MAS), and tocilizumab was used more frequently in patients with persistent type (p=0.000 and p=0.003). The most frequent switch rate was seen in patients receiving anakinra (n=40/68, 58.8%), and it was most frequently switched to canakinumab (n=32/40, 80%). Better physician's global assessment scores were achieved in patients treated with anakinra in Month 3, compared to other treatments (p=0.04). CONCLUSIONS: The results of our study support the efficacy of biological drugs in particular anti-IL-1 and anti-IL-6 drugs, in the treatment of SJIA. These treatments resulted in improvement in activity of disease and provide a considerable decrease in the frequency of MAS.

Risk of tuberculosis in children with rheumatologic diseases treated with biological agents: A cross-sectional cohort study.

Objectives: This study aimed to evaluate the risk of tuberculosis (TB) disease in children receiving biological agents for rheumatologic diseases, focusing on appropriate screening tests in a high-priority country for TB control. Patients and methods: One hundred nine children (56 females, 53 males; range, 3.4 to 16.2 years) who received any biological agent for rheumatologic diseases for more than two years between May 2012 and October 2021 were included in this retrospective study. Patients were screened for TB infection using tuberculin skin test (TST) or interferon-gamma release assay (IGRA). Following the initial evaluation, patients were clinically examined for TB every three months by a comprehensive medical history and physical examination, and every 12 months using TST or IGRA. Results: At the initiation of the biological agent, the patients' mean age was 12.4±4.5 years. The average follow-up duration was 3.6±1.3 years (range, 2.6 to 10.2 years) for patients treated with biological agents. Each patient had a documented Bacillus Calmette-Guérin vaccination. Before the initiating of therapy, TST was performed alone in 45 (41.3%) patients and in combination with IGRA in 64 (58.7%) patients. In the 64 patients who underwent both TST and IGRA, IGRA revealed nine (14.1%) positive results. Six (66.7%) of these nine patients, however, had negative baseline TST. Four (7.3%) of the 55 individuals whose initial IGRA results were negative also had positive TST results. Overall, no TB disease was observed after a follow-up period. Conclusion: This study reveals that biological agents were not associated with an increased risk of TB disease in closely monitored children. Additionally, the concomitant use of TST and IGRA for screening of TB is reasonable in patients receiving biological agents.

The clinical course of SARS-CoV-2 infection among children with rheumatic disease under biologic therapy: a retrospective and multicenter study.

The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course of COVID-19 on children with underlying rheumatologic diseases have not been fully demonstrated. To evaluate the course of COVID-19 infection in patients with rheumatic disease receiving bDMARD treatment. This was a retrospective, multicenter study conducted in pediatric patients infected by SARS-CoV-2 and under bDMARDs therapy. The study population consisted of 113 patients (72 female/41 male). The mean age of the patients was 12.87 ± 4.69 years. The primary diagnosis of the cohort was as follows: 63 juvenile idiopathic arthritis, 35 systemic autoinflammatory diseases, 10 vasculitides, and five cases of connective tissue diseases. The mean duration of the primary disease was 4.62 ± 3.65 years. A total of 19 patients had additional comorbid diseases. Thirty-five patients were treated with canakinumab, 25 with adalimumab, 18 with etanercept, 10 with infliximab, nine with tocilizumab, six with rituximab, four with anakinra, three with tofacitinib, and one with abatacept. The median exposure time of the biological drug was 13.5 months. Seventy-one patients had symptomatic COVID-19, while 42 were asymptomatic. Twenty-four patients required hospitalization. Five patients presented with MIS-C. The hospitalized patients were younger and had a shorter duration of rheumatic disease compared to ambulatory patients, although the difference was not statistically significant. Steroid usage, presence of fever, and dyspnea were more common among the hospitalized patients. A worsening in the course of both COVID-19 and current disease was not noticed under bDMARDs, however, to end with a strong conclusion multicentric international studies are required.

The effects of 8-week water-running program on exercise capacity in children with juvenile idiopathic arthritis: a controlled trial.

Exercise capacity has been reported to be lower in children with Juvenile Idiopathic Arthritis (JIA). Therefore, the aim was to investigate the effects of an 8-week water-based exercise program on exercise capacity in children with JIA. Forty-two children with JIA were divided into two groups as: exercise group [n = 21, water-running, moderate-intensity exercise (60-70%), two times/week], and control group (n = 21, no additional treatment other than the prescribed medication). All children were assessed at baseline and post-intervention in terms of physical and disease-related characteristics, pain at rest and in activity (visual analog scale), range of motion (Escola Paulista de Medicina Range of Motion Scale), aerobic exercise capacity (cycle ergometer), and anaerobic exercise capacity (Wingate Test). Anaerobic exercise capacity was found to be improved in the exercise group [baseline: 5.54 W/kg (IQR 25/75: 4.07/6.88 W/kg) vs. post-intervention: 6.0 W/kg (IQR 25/75: 4.8/7.4 W/kg), p = 0.002], while no improvements were observed in the control group [baseline: 5.29 W/kg (IQR 25/75: 4.75/5.85 W/kg) vs. post-intervention: 5.5 watts/kg (IQR 25/75: 5.0/6.1 W/kg), p = 0.076]. The amount of the changes related to anaerobic exercise capacity were higher in the exercise group [exercise group: 0.6 W/kg (IQR 25/75: 0.3/1.3 W/kg) vs. control group: 0.2 W/kg (IQR 25/75: - 0.1/0.5 W/kg), p = 0.024]. No changes were detected related to aerobic exercise capacity in any of the groups (p > 0.05). An 8-week water-running program might be beneficial to improve anaerobic exercise capacity, but it is not enough to improve the aerobic exercise capacity in children with JIA.

Evaluation of Restless Legs syndrome and growing pains in children with familial Mediterranean f.

Altug-Gücenmez Ö, Makay B, Kaçar A, Ünsal E. Evaluation of restless legs syndrome and growing pains in children with familial Mediterranean fever. Turk J Pediatr 2018; 60: 159-164. Growing pains (GP) and restless leg syndrome (RLS) are one of the frequently seen pain syndromes of childhood. These two pain syndromes -GP and RLS- may be confused with exertional leg pain (ELP) of familial Mediterranean fever (FMF). The aim of this study was to evaluate the frequency of fulfilling the criterion for GP and RLS among children with FMF. Sixty FMF patients and 70 healthy controls were enrolled. Clinical and demographic data of patients were recorded. A questionnaire including the symptoms of GP and RLS were applied to all participants and their parents. Twenty-seven patients (45%) had ELP. Ten FMF patients and 10 healthy children fulfilled GP criteria. There was not a significant difference between patients and controls regarding GP. Three FMF patients and 7 healthy controls fulfilled RLS criteria. There was not a significant difference between two groups regarding RLS. Two out of 27 FMF patients with ELP had RLS while one out of 33 FMF patients without ELP had RLS. Eight out of 27 FMF patients with ELP fulfilled GP criteria while two out of 33 FMF patients without ELP fulfilled GP criteria (p=0.01). There was a significant inverse correlation with FMF severity score and GP (p=0.003 and r=-0.376). There was not a significant association with FMF severity score and RLS. This study suggested that GP and RLS are not more common in pediatric FMF patients than their healthy peers.