Objective Systemic juvenile idiopathic arthritis (JIA) is associated with a recently recognized, albeit poorly defined and characterized, lung disease (LD). The objective of this study was to describe the clinical characteristics, risk factors, and histopathologic and immunologic features of this novel inflammatory LD associated with systemic JIA (designated SJIA‐LD). Methods Clinical data collected since 2010 were abstracted from the medical records of patients with systemic JIA from the Cincinnati Children's Hospital Medical Center. Epidemiologic, cellular, biochemical, genomic, and transcriptional profiling analyses were performed. Results Eighteen patients with SJIA‐LD were identified. Radiographic findings included diffuse ground‐glass opacities, subpleural reticulation, interlobular septal thickening, and lymphadenopathy. Pathologic findings included patchy, but extensive, lymphoplasmacytic infiltrates and mixed features of pulmonary alveolar proteinosis (PAP) and endogenous lipoid pneumonia. Compared to systemic JIA patients without LD, those with SJIA‐LD were younger at the diagnosis of systemic JIA (odds ratio [OR] 6.5, P = 0.007), more often had prior episodes of macrophage activation syndrome (MAS) (OR 14.5, P < 0.001), had a greater frequency of adverse reactions to biologic therapy (OR 13.6, P < 0.001), and had higher serum levels of interleukin‐18 (IL‐18) (median 27,612 pg/ml versus 5,413 pg/ml; P = 0.047). Patients with SJIA‐LD lacked genetic, serologic, or functional evidence of granulocyte–macrophage colony‐stimulating factor pathway dysfunction, a feature that is typical of familial or autoimmune PAP. Moreover, bronchoalveolar lavage (BAL) fluid from patients with SJIA‐LD rarely demonstrated proteinaceous material and had less lipid‐laden macrophages than that seen in patients with primary PAP (mean 10.5% in patients with SJIA‐LD versus 66.1% in patients with primary PAP; P < 0.001). BAL fluid from patients with SJIA‐LD contained elevated levels of IL‐18 and the interferon‐γ–induced chemokines CXCL9 and CXCL10. Transcriptional profiling of the lung tissue from patients with SJIA‐LD identified up‐regulated type II interferon and T cell activation networks. This signature was also present in SJIA‐LD human lung tissue sections that lacked substantial histopathologic findings, suggesting that this activation signature may precede and drive the lung pathology in SJIA‐LD. Conclusion Pulmonary disease is increasingly detected in children with systemic JIA, particularly in association with MAS. This entity has distinct clinical and immunologic features and represents an uncharacterized inflammatory LD.

Objective. Juvenile fibromyalgia syndrome (FMS)is a chronic musculoskeletal pain disorder in children and adolescents for which there are no evidence-based treatments. The objective of this multisite, single-blind, randomized clinical trial was to test whether cognitivebehavioral therapy (CBT) was superior to fibromyalgia (FM) education in reducing functional disability, pain, and symptoms of depression in juvenile FMS.Methods. Participants were 114 adolescents (ages 11-18 years) with juvenile FMS. After receiving stable medications for 8 weeks, patients were randomized to either CBT or FM education and received 8 weekly individual sessions with a therapist and 2 booster sessions. Assessments were conducted at baseline, immediately following the 8-week treatment phase, and at 6-month followup.Results. The majority of patients (87.7%) completed the trial per protocol. Intent-to-treat analyses showed that patients in both groups had significant reductions in functional disability, pain, and symptoms of depression at the end of the study, and CBT was significantly superior to FM education in reducing the primary outcome of functional disability (mean baseline to end-of-treatment difference between groups 5.39 [95% confidence interval 1.57, 9.22]). Reduction in symptoms of depression was clinically significant for both groups, with mean scores in the range of normal/ nondepressed by the end of the study. Reduction in pain was not clinically significant for either group (<30% decrease in pain). There were no study-related adverse events.Conclusion. In this controlled trial, CBT was found to be a safe and effective treatment for reducing functional disability and symptoms of depression in adolescents with juvenile FMS.Juvenile fibromyalgia syndrome (FMS) is a chronic pain condition that is estimated to affect 2-7% of school-age children (1-3), primarily adolescent girls. As in adult fibromyalgia (FM), juvenile FMS is characterized by persistent and widespread musculoskeletal pain, sleep difficulty, fatigue, and mood disturbances (3). Patients with juvenile FMS experience substantial impairment in physical, school, social, and emotional functioning (4-6), and the majority of them continue to have ongoing symptoms and functional disability into ClinicalTrials.gov identifier: NCT00086047.

Patients with cSLE were only modestly adherent to HCQ and clinic visits. CTMR may be effective for improving visit adherence among adolescents and young adults with cSLE, but it does not improve adherence to HCQ.

WHAT'S KNOWN ON THIS SUBJECT: Juvenile-onset fibromyalgia (JFM) is a poorly understood chronic pain condition, typically identified in adolescence and accompanied by physical and social impairment and mood difficulties. There are no long-term studies on the prognosis of adolescents with JFM into adulthood. WHAT THIS STUDY ADDS:This prospective study demonstrated that pain and other symptoms persisted into adulthood for .80% of JFM patients, with associated impairments in physical functioning and mood. At follow-up, one-half of the sample met full criteria for adult fibromyalgia. abstractOBJECTIVE: This prospective longitudinal study examined the long-term physical and psychosocial outcomes of adolescents with juvenile-onset fibromyalgia (JFM), compared with healthy control subjects, into early adulthood.METHODS: Adolescent patients with JFM initially seen at a pediatric rheumatology clinic (n = 94) and age-and gender-matched healthy control subjects (n = 33) completed online measures of demographic characteristics, pain, physical functioning, mood symptoms, and health care utilization at ∼6 years' follow-up (mean age: 21 years). A standard in-person tender-point examination was conducted. RESULTS:Patients with JFM had significantly higher pain (P , .001), poorer physical function (P , .001), greater anxiety (P , .001) and depressive symptoms (P , .001), and more medical visits (P , .001) than control subjects. The majority (.80%) of JFM patients continued to experience fibromyalgia symptoms into early adulthood, and 51.1% of the JFM sample met American College of Rheumatology criteria for adult fibromyalgia at follow-up. Patients with JFM were more likely than control subjects to be married and less likely to obtain a college education.CONCLUSIONS: Adolescent patients with JFM have a high likelihood of continued fibromyalgia symptoms into young adulthood. Those who met criteria for fibromyalgia in adulthood exhibited the highest levels of physical and emotional impairment. Emerging differences in educational attainment and marital status were also found in the JFM group. JFM is likely to be a long-term condition for many patients, and this study for the first time describes the wide-ranging impact of JFM on a variety of physical and psychosocial outcomes that seem to diverge from their same-age peers.

A recent randomized multi-site clinical trial found that cognitive-behavioral therapy (CBT) was significantly more effective than fibromyalgia education (FE) in reducing functional disability in adolescents with juvenile fibromyalgia (JFM). The primary objective of this study was to examine the psychological processes of CBT effectiveness by evaluating changes in pain coping, catastrophizing, and coping efficacy and test these changes as mediators of continued improvements in functional disability and depressive symptoms at 6-month follow-up. One hundred adolescents (11–18 years old) with JFM completed the clinical trial. Coping, catastrophizing, and coping efficacy (Pain Coping Questionnaire) and the outcomes of functional disability (Functional Disability Inventory) and depressive symptoms (Children’s Depression Inventory) were measured at baseline, post-treatment, and 6-month follow-up. Participants in both conditions showed significant improvement in coping, catastrophizing, and efficacy by the end of the study, but significantly greater improvements were found immediately following treatment for those who received CBT. Treatment gains were maintained at follow-up. Baseline to post-treatment changes in coping, catastrophizing, and efficacy were not found to mediate improvements in functional disability or depressive symptoms from post-treatment to follow-up. Future directions for understanding mechanisms of CBT effectiveness in adolescents with chronic pain are discussed.

Objectives Adolescents with juvenile fibromyalgia (JFM) are typically sedentary despite recommendations for physical exercise, a key component of pain management. Interventions such as cognitive behavioral therapy (CBT) are beneficial but do not improve exercise participation. The objective of this study was to obtain preliminary information about the feasibility, safety, and acceptability of a new intervention - Fibromyalgia Integrative Training for Teens (FIT Teens), which combines CBT with specialized neuromuscular exercise training modified from evidence-based injury prevention protocols. Methods Participants were 17 adolescent females (ages 12–18) with JFM. Of these, 11 completed the 8-week (16-session) FIT Teens program in a small-group format with 3–4 patients per group. Patients provided detailed qualitative feedback via individual semi-structured interviews after treatment. Interview content was coded using thematic analysis. Interventionist feedback about treatment implementation was also obtained. Results The intervention was found to be feasible, well-tolerated, and safe for JFM patients. Barriers to enrollment (50% of those approached) included difficulties with transportation or time conflicts. Treatment completers enjoyed the group format and reported increased self-efficacy, strength, and motivation to exercise. Participants also reported decreased pain and increased energy levels. Feedback from participants and interventionists was incorporated into a final treatment manual to be used in a future trial. Discussion Results of this study provided initial support for the new FIT Teens program. An integrative strategy of combining pain coping skills via CBT enhanced with tailored exercise specifically designed to improve confidence in movement and improving activity participation holds promise in the management of JFM.

ObjectivesMusculoskeletal ultrasonography (US) has the potential to be an important tool in the assessment of disease activity in childhood arthritides. To assess pathology, clear definitions for synovitis need to be developed first. The aim of this study was to develop and validate these definitions through an international consensus process. MethodsThe decision on which US techniques to use, the components to be included in the Arthritis Care & ResearchThis article is protected by copyright. All rights reserved. Sonographic Definitions for Synovitis in Children 3 Significance and InnovationsMusculoskeletal Ultrasonography is an important tool for the clinical assessment and research in childhood arthritides Precise definitions for synovitis on ultrasonography in children are an essential prerequisite for the reliable use of this technology in the pediatric age group Ultrasonographic definitions for synovitis in children were developed and validated for the first time through an international consensus process

The 2010 ACR measure appears to be a valuable tool for the identification of JFM. However, a slight modification to the 2010 ACR measure and inclusion of a clinical exam is recommended.