Starting in 2015, pediatric rheumatology fellowship training programs were required by the Accreditation Council for Graduate Medical Education to assess fellows' academic performance within 21 subcompetencies falling under six competency domains. Each subcompetency had four or five milestone levels describing developmental progression of knowledge and skill acquisition. Milestones were standardized across all pediatric subspecialties. As part of the Milestones 2.0 revision project, the Accreditation Council for Graduate Medical Education convened a workgroup in 2022 to write pediatric rheumatology-specific milestones. Using adult rheumatology's Milestones 2.0 as a starting point, the workgroup revised the patient care and medical knowledge subcompetencies and milestones to reflect requirements and nuances of pediatric rheumatology care. Milestones within four remaining competency domains (professionalism, interpersonal and communication skills, practice-based learning and improvement, and systems-based practice) were standardized across all pediatric subspecialties, and therefore not revised. The workgroup created a supplemental guide with explanations of the intent of each subcompetency, 25 in total, and examples for each milestone level. The new milestones are an important step forward for competency-based medical education in pediatric rheumatology. However, challenges remain. Milestone level assignment is meant to be informed by results of multiple assessment methods. The lack of pediatric rheumatology-specific assessment tools typically result in clinical competency committees determining trainee milestone levels without such collated results as the foundation of their assessments. Although further advances in pediatric rheumatology fellowship competency-based medical education are needed, Milestones 2.0 importantly establishes the first pediatric-specific rheumatology Milestones to assess fellow performance during training and help measure readiness for independent practice.

IntroductionThe Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a North American learning health network focused on improving outcomes of children with juvenile idiopathic arthritis (JIA). JIA is a chronic autoimmune disease that can lead to morbidity related to persistent joint and ocular inflammation. PR-COIN has a shared patient registry that tracks twenty quality measures including ten outcome measures of which six are related to disease activity. The network's global aim, set in 2021, was to increase the percent of patients with oligoarticular or polyarticular JIA that had an inactive or low disease activity state from 76% to 80% by the end of 2023.MethodsTwenty-three hospitals participate in PR-COIN, with over 7,200 active patients with JIA. The disease activity outcome measures include active joint count, physician global assessment of disease activity, and measures related to validated composite disease activity scoring systems including inactive or low disease activity by the 10-joint clinical Juvenile Arthritis Disease Activity Score (cJADAS10), inactive or low disease activity by cJADAS10 at 6 months post-diagnosis, mean cJADAS10 score, and the American College of Rheumatology (ACR) provisional criteria for clinical inactive disease. Data is collated to measure network performance, which is displayed on run and control charts. Network-wide interventions have included pre-visit planning, shared decision making, self-management support, population health management, and utilizing a Treat to Target approach to care.ResultsFive outcome measures related to disease activity have demonstrated significant improvement over time. The percent of patients with inactive or low disease activity by cJADAS10 surpassed our goal with current network performance at 81%. Clinical inactive disease by ACR provisional criteria improved from 46% to 60%. The mean cJADAS10 score decreased from 4.3 to 2.6, and the mean active joint count declined from 1.5 to 0.7. Mean physician global assessment of disease activity significantly improved from 1 to 0.6.ConclusionsPR-COIN has shown significant improvement in disease activity metrics for patients with JIA. The network will continue to work on both site-specific and collaborative efforts to improve outcomes for children with JIA with attention to health equity, severity adjustment, and data quality.

Mental health, which encompasses psychological and emotional well-being, is increasingly being recognized as an important aspect of overall health in youth with chronic disease. Particularly in this time of the mental health crisis in children due in large part to the coronavirus disease 2019 (COVID-19) pandemic,1 with evidence of adverse effects on managing rheumatologic disease,2 addressing mental health issues has become critical. In the pediatric rheumatology population, mental health problems have been shown to affect quality of life, educational attainment, and disease-related outcomes such as medication adherence, healthcare utilization, and transition.3 Although patients with juvenile idiopathic arthritis (JIA) represent the largest disease group in pediatric rheumatology, there are gaps in our understanding of mental health effects in JIA, and research to optimize mental health for these patients is needed. In this issue of The Journal of Rheumatology , Li et al present the results of an interesting cross-sectional study of children with JIA aged 12 to 18 years, examining rates of depressive and anxiety symptoms, and their association with demographic and disease measures.4 The sample included 80 Canadian adolescents who presented to a tertiary care pediatric hospital and had JIA diagnoses of varying subtypes for at least 6 months. Based on clinical threshold scores on the self-reported Revised Child Anxiety and Depression Scale (RCADS), the numbers of youth endorsing symptoms consistent with a mental health disorder were described according to 6 subscales (ie, separation anxiety, social phobia, generalized anxiety, panic disorder, obsessive-compulsive disorder, and major depressive disorder). Differences in demographic and disease-related factors (eg, patient-reported disease activity, physician global assessment of disease activity [PGA], widespread pain, joint and enthesitis count) between those with and without clinically elevated mental health symptoms were also explored. Results indicated a recruitment rate of 98%, and a patient sample consisting of … Address correspondence to Dr. A. Knight, Hospital for Sick Children, Division of Rheumatology, Department of Pediatrics, 555 University Ave, Toronto, ON M5G 1X8, Canada. Email: andrea.knight{at}sickkids.ca.

Objective Describe anxiety and depressive symptoms in children with juvenile idiopathic arthritis (JIA) using Patient Reported Outcomes Measurement Information System (PROMIS) measures and evaluate potential correlations with disease manifestations. Methods We performed a cross-sectional study of children with JIA and a parent proxy who completed PROMIS measures on depression, anxiety, stress, and pain. The Childhood Health Assessment Questionnaire (CHAQ) measured mobility, and the clinical Juvenile Arthritis Disease Activity Score in 10 joints (cJADAS10) measured disease activity. Results Eighty-four patients completed the study. Demographic median values included age (14 yrs), disease duration (4.73 yrs), CHAQ score (0), total active joint count (0), and cJADAS10 (2). Using cJADAS10, 57 patients (70%) had inactive or low disease activity. Mean PROMIS t-scores for depressive and anxiety symptoms were lower in children with JIA compared to the reference population (P < 0.0001). Nineteen patients (23%) had moderate to severe symptoms of anxiety and/or depression. Age and CHAQ score (mobility) correlated with depressive symptoms (r = 0.36, P =0.0008 and r = 0.32, P = 0.0029, respectively) but not anxiety. Depressive and anxiety symptoms correlated with pain (r = 0.64 and r = 0.47, respectively, P < 0.0001) and stress (r = 0.79 and r = 0.75, respectively, P < 0.0001) but not with sex, JIA subtype, disease duration, or disease activity. Conclusion Approximately one-quarter of children with JIA reported moderate to severe symptoms of anxiety and depression. These symptoms are associated with pain and stress, but they are not associated with other disease manifestations. Understanding how mental health symptoms and JIA affect each other is necessary in order to improve patient outcomes and provide well-rounded care.

Depression and anxiety are prevalent in children with rheumatologic diseases, including juvenile idiopathic arthritis (JIA). However, prevalence rates and the relationship with disease outcomes, including quality of life are conflicting in the early literature. To review the current literature, determine gaps in our knowledge, and identify areas in need of further investigation, we conducted a systematic review of studies examining depression and anxiety symptoms among children with JIA and the impact these symptoms may have on disease outcomes and quality of life. Six electronic databases were searched up until January 2019. Of 799 potential articles, 60 articles were included with the main focus on 28 articles from 2009 to 2019, to concentrate on the most current evidence. We found that JIA patients experience symptoms of depression and anxiety similar to other childhood chronic diseases and at higher rates than in healthy children. Patients who experience these symptoms have worse quality of life, with some evidence pointing to depression and anxiety symptoms having a greater impact on quality of life than other disease features, such as active joint count. Family members of JIA patients experience high rates of anxiety and depression symptoms which may impact their child's mental health and pain symptoms related to JIA. Conflicting reports of associations between depression/anxiety symptoms and disease features/disease outcomes and a paucity of longitudinal studies investigating the impact of treatment on mental health symptoms indicate areas in need of further research to effectively identify patients at greatest risk of depression and anxiety and to better understand how to treat and prevent these symptoms in youth with JIA. Family mental health should also be considered in investigations concerning mental health and disease outcomes of children with JIA.

Advances in genetic techniques have led to the discovery of new autoinflammatory syndromes in the last three years. The purpose of this review is to discuss novel autoinflammatory syndromes including disease mechanisms, characteristics, and treatments. We will also discuss recent findings regarding the pathophysiology of well-known autoinflammatory disorders.