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BACKGROUND: To report baseline characteristics, patient reported outcomes and treatment of children with Juvenile Dermatomyositis (JDM) in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. METHODS: Children newly diagnosed with JDM were enrolled in the CARRA Registry from 41 pediatric rheumatology centers. Baseline patient demographics, disease characteristics, assessments, patient reported outcome and treatments were recorded. RESULTS: In the first year, 119 JDM participants were enrolled. Most were female (63.4%), and white (72.3%) with a median diagnosis age 8.0 years (IQR 4.0-11.5), and median age of disease onset 7.0 years (IQR 3.5-7.5). They had characteristic rashes (92.4%), elevated muscle enzymes (83.2%), physician global score 4.0 (IQR 2.5-5.0) and manual muscle testing score 63.5 (IQR 51.0-75.0). Calcinosis (3.4%) and interstitial lung disease (

Background The Internet is a significant source of medical information and is now being shown to be an important conduit for delivering various health-related interventions. Objective This paper aimed to examine the utility and impact of an Internet intervention for childhood encopresis as part of standard medical care in a “real world” setting. Methods Patients diagnosed with encopresis were given a Web-based information prescription to use an Internet intervention for pediatric encopresis. A total of 22 families utilized the intervention between July 2004 and June 2006. A chart review and phone interview were undertaken to collect user characteristics; defecation-related information, including frequency of soiling, bowel movements (BMs) in the toilet, and amount of pain associated with defecation; and information on computer/Internet usage. Three questionnaires were used to examine the utility of, impact of, and adherence to the Internet intervention. Program utilization was obtained from a data tracking system that monitored usage in real time. Results Overall, parents rated the Internet intervention as enjoyable, understandable, and easy to use. They indicated that the Internet intervention positively affected their children, decreasing overall accidents and increasing child comfort on the toilet at home. Of the 20 children who initially reported fecal accidents, 19 (95%) experienced at least a 50% improvement, with a reduction of accident frequency from one fecal accident per day to one accident per week. Although it is not clear whether this improvement is directly related to the use of the Internet intervention, patient feedback suggests that the program was an important element, further establishing Internet interventions as a viable and desirable addition to standard medical care for pediatric encopresis. Conclusions To our knowledge, this is the first time a pediatric Internet intervention has been examined as part of a “real world” setting. This is an important step toward establishing Internet interventions as an adjunctive component to treatment of pediatric patients in a clinical setting, particularly given the positive user feedback, possible cost savings, and significant potential for large-scale dissemination.

Objective: Myositis‐associated autoantibodies (MAAs) have been associated with overlap myositis, certain disease manifestations such as interstitial lung disease (ILD), and worse prognosis in the idiopathic inflammatory myopathies. MAAs overall remain largely uncharacterized in patients with juvenile‐onset myositis. Moreover, it is unknown whether the number of MAAs is associated with disease severity. Methods: Patients with juvenile myositis in cross‐sectional natural history studies who underwent testing for myositis autoantibodies were included. Demographics, myositis autoantibodies, clinical characteristics, medications received, and outcomes of those with and without MAAs were compared. Multivariable logistic regression was performed to determine whether the number of MAAs detected was associated with severe disease features. Results: Among 551 patients, 36% had an MAA and 13% had more than one MAA. Among those who were MAA positive, there was a higher frequency of overlap myositis (18% vs 5.9%, P < 0.001). MAA positivity was associated with certain clinical features, including Raynaud phenomenon (odds ratio [OR] 2.44, 95% confidence interval [CI] 1.41–4.28) and ILD (OR 3.43, 95% CI 1.75–6.96), as well as a chronic disease course (OR 1.72, 95% CI 1.10–2.72) and mortality (OR 3.76, 95% CI 1.72–8.43). The number of MAAs was also associated with mortality (OR 1.83, 95% CI 1.16–2.86). Conclusion: MAAs were prevalent in a large cohort of patients with juvenile myositis. ILD, refractory disease, and mortality were associated with MAA positivity. Prospective studies are needed to determine whether early detection of MAAs may lead to improved outcomes for patients with juvenile myositis. [ABSTRACT FROM AUTHOR]

Objective: Patient‐Reported Outcomes Measurement Information System (PROMIS) measures can be administered via computerized adaptive testing (CAT) or fixed short forms (FSFs), but the empirical benefits of CAT versus FSFs are unknown in juvenile myositis (JM). The present study was undertaken to assess whether PROMIS CAT is feasible, precise, correlated with FSFs, and less prone to respondent burden and floor/ceiling effects than FSFs in JM. Methods: Patients 8–17 years of age (self‐report and parent proxy) and parents of patients 5–7 years of age (only parent proxy) completed PROMIS fatigue, pain interference, upper extremity function, mobility, anxiety, and depressive symptoms measures. Pearson correlations, paired t‐tests, and Cohen's d were calculated between PROMIS CAT and FSFs. McNemar's test assessed floor/ceiling effects between CAT and FSFs. Precision and respondent burden were examined across the T score range. Results: Data from 67 patient–parent dyads were analyzed. CAT and FSF mean scores did not significantly differ except in parent proxy anxiety and fatigue (effect size 0.23 and 0.19, respectively). CAT had less pronounced floor/ceiling effects at the less symptomatic extreme in all domains except self‐report anxiety. Increased item burden and higher SEs were seen in less symptomatic scorers for CAT. Modified stopping rules limiting CAT item administration did not decrease precision. Conclusion: PROMIS CAT appears to be feasible and correlated with FSFs. CAT had less pronounced floor/ceiling effects, allowing detection of individual differences in less symptomatic patients. Modified stopping rules for CAT may decrease respondent burden. CAT can be considered for long‐term follow‐up of JM patients. [ABSTRACT FROM AUTHOR]

Objective: To provide evidence‐based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists. Methods: Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question. We used the Grading of Recommendations, Assessment, Development and Evaluation method to assess the quality of evidence and formulate recommendations. Each recommendation required consensus from at least 70% of the Voting Panel. Results: We present 1 good practice statement, 11 recommendations, and 1 ungraded position statement to guide the management of KD and clinical scenarios of suspected KD. These recommendations for KD are focused on situations in which input from rheumatologists may be requested by other managing specialists, such as in cases of treatment‐refractory, severe, or complicated KD. The good practice statement affirms that all patients with KD should receive initial treatment with intravenous immunoglobulin (IVIG). In addition, we developed 7 strong and 4 conditional recommendations for the management of KD or suspected KD. Strong recommendations include prompt treatment of incomplete KD, treatment with aspirin, and obtaining an echocardiogram in the setting of unexplained macrophage activation syndrome or shock. Conditional recommendations include use of IVIG with other adjuvant agents for patients with KD and high‐risk features of IVIG resistance and/or coronary artery aneurysms. These recommendations endorse minimizing risk to the patient by using established therapy promptly at disease onset and identifying situations in which adjunctive therapy may be warranted. Conclusion: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and use of echocardiography in patients with suspected or confirmed KD. [ABSTRACT FROM AUTHOR]

This topical review summarizes recent literature on mental health symptoms experienced by children diagnosed with rheumatologic conditions including childhood-onset systemic lupus erythematosus (cSLE), juvenile idiopathic arthritis (JIA), and juvenile dermatomyositis (JDM). Studies, while limited, generally indicate that anxiety and depressive symptoms may be more common among children diagnosed with rheumatologic conditions than non-chronically ill children. Although the rates of clinically significant symptoms are not consistently reported across studies, overall anxiety and depressive symptom rates in cSLE vary between 34%–37% and 6.7%–59%, respectively. A recent systematic review of JIA suggests between 7% and 64% of participants experienced elevated anxiety, and between 7% and 36% of participants reported clinically significant depressive symptoms. Approximately 40% of youth with JDM may experience general psychological distress, but more research is needed. In the available literature, there is mixed support for higher rates of anxiety in JIA as compared to cSLE, and higher rates of depressive symptoms in cSLE as compared to JIA, whereas mental health functioning in JDM is less well understood. Mental health functioning in youth with rheumatologic conditions may be related to increased disease-related impairment. Using consistent mental health screening measures with clinically validated cutoffs would enhance insight into the frequency and impact of anxiety and depressive symptoms experienced. Knowledge would also be enhanced by conducting studies with ethnically representative samples to identify potential disparities in care. An improved understanding of mental health functioning in pediatric patients presenting for rheumatologic care may inform the development and testing of tailored and effective treatments. [ABSTRACT FROM AUTHOR]

Objective: To assess parent perspectives regarding the emotional health impact of juvenile myositis (JM) on patients and families, and to assess preferences for emotional health screening and interventions. Methods: Parents of children and young adults with JM were purposively sampled for participation in focus groups at the Cure JM Foundation National Family Conference in 2018. Groups were stratified by patient age group (6–12, 13–17, and 18–21 years), and conversations were audiorecorded, transcribed verbatim, and co‐coded via content analysis, with subanalysis by age group. A brief survey assessed preferences for specific emotional health interventions. Results: Forty‐five parents participated in 6 focus groups. Themes emerged within 2 domains: emotional challenges, and screening and interventions. Themes for emotional challenges comprised the impact of JM on: 1) patient emotional health, particularly depression and anxiety; 2) parent emotional health characterized by sadness, grief, anger, guilt, and anxiety; and 3) family dynamics, including significant sibling distress. Subanalysis revealed similar themes across age groups, but the theme of resiliency emerged specifically for young adults. Themes for emotional health screening and interventions indicated potential issues with patient transparency, several barriers to resources, the facilitator role of rheumatology providers, and preferred intervention modalities of online and in‐person resources, with survey responses most strongly supporting child/parent counseling and peer support groups. Conclusion: JM is associated with intense patient and family distress, although resiliency may emerge by young adulthood. Despite existing barriers, increasing access to counseling, peer support groups, and online resources with rheumatology facilitation may be effective intervention strategies. [ABSTRACT FROM AUTHOR]

Background: Standardized pediatric hospitalist and orthopaedic co-management of spinal fusion patients may improve quality processes and outcomes. This approach has not been studied in a general academic center. Objective: Estimate relative effects and feasibility of the interventions on quality outcomes, length of stay (LOS), catheter-acquired urinary tract infections (CAUTI), medication errors, and pain scores. Design and Setting: Retrospective cohort using interrupted time series, analyzing data from 83 patients aged 5 to 18 years admitted for posterior spinal fusion (PSF) in 2009 (N = 27), 2010 (N = 28), and 2011 (N = 28) on a children's service at a general academic tertiary care center. Interventions: Multimodal approach to standardizing pediatric PSF postoperative care with interdepartmental development of order sets, clinical care guidelines, and routine pediatric hospitalist co-management of all pediatric PSF patients. Measurements: Chi-square analysis of order set use, guideline use measured by proxy medication and documentation data. ANOVA for comparison of CA UTI and medication error rate and multivariate linear regression of LOS and pain scores. Results: Pediatric hospitalist co-management documentation increased from 64% to 80%. Guideline use increased from 40% to 79%, and order set use was < 15%. CAUTI and medication error ratios remained low. Adjusted mean LOS decreased by 0.8 days (p = 0.039, 95% CI 0.7, 1.1). Pain scores did not differ. Conclusion: Interdisciplinary, clinical guideline development and postoperative co-management significantly decreased hospital LOS in pediatric PSF patients. In a general academic medical center, this change may be attributed to a pediatric hospitalist academic team, a universal co-management process with well-communicated roles, and a pediatric hospital-based physician development of and adherence to standardized practice. [ABSTRACT FROM AUTHOR]

Objective: Localized scleroderma can negatively affect children's quality of life (QoL), but predictors of impact have not been well described. We sought to identify predictors of QoL impact in juvenile localized scleroderma patients.Methods: We analyzed longitudinal data from a single-center cohort of juvenile localized scleroderma patients, using hierarchical generalized linear modeling (HGLM) to identify predictors of QoL impact. HGLM is useful for nested data and allows for evaluation of both time-variant and time-invariant predictors.Results: The number of extracutaneous manifestations (ECMs; e.g., joint contracture and hemifacial atrophy) and female sex predicted negative QoL impact, defined as a Children's Dermatology Life Quality Index score >1 (P = 0.019 for ECMs and P = 0.002 for female sex). As the time since the initial visit increased, the odds of reporting a negative QoL impact decreased (P < 0.001).Conclusion: Our results suggest that ECMs, sex, and time since initial visit are more predictive of QoL impact in localized scleroderma than cutaneous features. Further study is required to determine which ECMs have the most impact on QoL, which factors underlie sex differences in QoL in localized scleroderma, and why increasing the time since the initial visit appears to be protective. An improved understanding of predictors of QoL impact may allow for the identification of patients at risk of poorer outcomes and for the tailoring of treatment and psychosocial support. [ABSTRACT FROM AUTHOR]