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 Localized scleroderma (LS) can negatively affect children’s quality of life (QoL), but predictors of impact are not well described. We sought to identify predictors of QoL impact in pediatric LS patients. Methods We analyzed longitudinal data from a single-center cohort of pediatric LS 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, hemifacial atrophy) and female gender predicted negative QoL impact, defined as Children’s Dermatology Life Quality Index (CDLQI) score > 1 (p = 0.019, p = 0.002 respectively). As time from initial visit increased, the odds of reporting negative QoL impact decreased (p < 0.001). Conclusion Our results suggest that ECMs, gender, and time from initial visit are more predictive of QoL impact in LS than cutaneous features. Further study is required to determine which ECMs have the most impact on QoL, which factors underlie gender differences in QoL in LS, and why increasing time from initial visit appears to be protective. Improved understanding of predictors of QoL impact may allow for identification of patients at risk of poorer outcomes and tailoring of treatment and psychosocial support.

BackgroundThe prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960’s with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011–2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA).MethodsThe JDM biologic study group developed a survey on the CARRA member experience using biologics for Juvenile DM utilizing Delphi consensus methods in 2011–2012. The survey was completed online by the CARRA members interested in JDM in 2012. A second survey was similarly developed that provided more opportunity to describe their experiences with biologics in JDM in detail and was completed by CARRA members in Feb 2013. During three CARRA meetings in 2013–2015, nominal group techniques were used for achieving consensus on the current choices of biologic drugs. A final survey was performed at the 2016 CARRA meeting.ResultsOne hundred and five of a potential 231 pediatric rheumatologists (42%) responded to the first survey in 2012. Thirty-five of 90 had never used a biologic for Juvenile DM at that time. Fifty-five of 91 (denominators vary) had used biologics for JDM in their practice with 32%, 5%, and 4% using rituximab, etanercept, and infliximab, respectively, and 17% having used more than one of the three drugs. Ten percent used a biologic as monotherapy, 19% a biologic in combination with methotrexate (mtx), 52% a biologic in combination with mtx and corticosteroids, 42% a combination of a biologic, mtx, corticosteroids (steroids), and an immunosuppressive drug, and 43% a combination of a biologic, IVIG and mtx. The results of the second survey supported these findings in considerably more detail with multiple combinations of drugs used with biologics and supported the use of rituximab, abatacept, anti-TNFα drugs, and tocilizumab in that order. One hundred percent recommended that CARRA continue studying biologics for JDM. The CARRA meeting survey in 2016 again supported the study and use of these four biologic drug groups.ConclusionsOur CARRA JDM biologic work group developed and performed three surveys demonstrating that pediatric rheumatologists in North America have been using multiple biologics for refractory JDM in numerous scenarios from 2011 to 2016. These survey results and our consensus meetings determined our choice of four biologic therapies (rituximab, abatacept, tocilizumab and anti-TNFα drugs) to consider for refractory JDM treatment when indicated and to evaluate for comparative effectiveness and safety in the future.Significance and InnovationsThis is the first report that provides a substantial clinical experience of a large group of pediatric rheumatologists with biologics for refractory JDM over five years.This experience with biologic thera...

There are significantly higher odds of cardiovascular and cerebrovascular comorbidities among inpatients with JDM, with adolescents, girls and racial/ethnic minorities being at highest risk.

Background Paediatric localized scleroderma (LS) can negatively impact healthrelated quality of life (HRQoL) by causing skin fibrosis, abnormal limb development, disfigurement, and side-effects from immunosuppressive treatment. Studies to date have rarely included qualitative data gathered directly from paediatric patients with LS. Objectives To assess the impact of LS on HRQoL among affected youth and their caregivers using qualitative description. Methods Youth with all subtypes of LS and their caregivers were purposively sampled to participate in age-appropriate focus groups (younger children, early adolescents, adolescents). Each group started with a drawing exercise followed by in-depth discussion of topics including skin symptoms (e.g. itch, pain, tightness), functional impairment, physical appearance, family and peer relationships, and treatment burden. Focus groups were transcribed verbatim and co-coded, with adjudication of differentially applied codes. The study findings were triangulated via comparison with adult reports and published literature. Results Eleven youth aged 9-16 years and 16 caregivers participated in three focus groups each. Major identified areas of impact included uncomfortable skin symptoms, physical functioning limitations, extracutaneous manifestations, body image, bullying and teasing, unwanted questioning from others, and treatment side-effects and burden. Conclusions This is the first qualitative study of HRQoL in LS to include all major LS subtypes. We identified domains of HRQoL impacted by LS, some of which replicate earlier findings and some of which were novel. As impact also changed with developmental stage, our findings support the need for ongoing, formal evaluation of HRQoL in children and adolescents with LS. What is already known about this topic? • Paediatric localized scleroderma (LS) negatively impacts health-related quality of life (HRQoL) via skin fibrosis, musculoskeletal and other extracutaneous manifestations from the disease process, and side-effects of systemic immunosuppression. • The full impact of LS and its treatment on HRQoL is incompletely understood, with only one published qualitative study of youth with LS, which was limited to facial involvement. • There are no qualitative studies of HRQoL in other LS subtypes to date.

One or more occurrences of ICD-9 code 710.3 is insufficient to support the diagnosis of dermatomyositis in the outpatient setting. However, ICD-9 710.3 codes appear to be valid in the inpatient setting.

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.

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