Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript. The Children’s Arthritis and Rheumatology Research Alliance (CARRA) is a North American consortium of pediatric rheumatology health care providers. CARRA members collaborated to determine consensus on typical treatments for JDM patients with skin findings without significant weakness, to develop CTPs for this subgroup of patients. We used a combination of Delphi surveys and nominal group consensus meetings to develop these CTPs. Consensus was reached on patient characteristics and outcome assessment, and CTPs were developed and finalized for patients with skin predominant JDM. Treatment option A included hydroxychloroquine alone, Treatment option B included hydroxychloroquine and methotrexate, and Treatment option C included hydroxychloroquine, methotrexate and corticosteroids. Three CTPs were developed for use in children with skin predominant JDM, which reflect typical treatment approaches. These are not considered to be specific recommendations or standard of care. Using the CARRA network and prospective data collection, we will be able to apply statistical methods in the future to allow comparisons of JDM patients following these consensus treatment plans.

With modern treatments, the effect of juvenile idiopathic arthritis (JIA) on growth may be less than previously reported. Our objective was to describe height, weight and body mass index (BMI) development in a contemporary JIA inception cohort. Canadian children newly-diagnosed with JIA 2005–2010 had weight and height measurements every 6 months for 2 years, then yearly up to 5 years. These measurements were used to calculate mean age- and sex-standardized Z-scores, and estimate prevalence and cumulative incidence of growth impairments, and the impact of disease activity and corticosteroids on growth. One thousand one hundred forty seven children were followed for median 35.5 months. Mean Z-scores, and the point prevalence of short stature (height < 2.5th percentile, 2.5% to 3.4%) and obesity (BMI > 95th percentile, 15.8% to 16.4%) remained unchanged in the whole cohort. Thirty-three children (2.9%) developed new-onset short stature, while 27 (2.4%) developed tall stature (>97.5th percentile). Children with systemic arthritis (n = 77) had an estimated 3-year cumulative incidence of 9.3% (95%CI: 4.3–19.7) for new-onset short stature and 34.4% (23–49.4) for obesity. Most children (81.7%) received no systemic corticosteroids, but 1 mg/Kg/day prednisone-equivalent maintained for 6 months corresponded to a drop of 0.64 height Z-scores (0.56–0.82) and an increase of 0.74 BMI Z-scores (0.56–0.92). An increase of 1 in the 10-cm physician global assessment of disease activity maintained for 6 months corresponded to a drop of 0.01 height Z-scores (0–0.02). Most children in this modern JIA cohort grew and gained weight as children in the general population. About 1 in 10 children who had systemic arthritis, uncontrolled disease and/or prolonged corticosteroid use, had increased risk of growth impairment.

An adolescent with a chronic condition must prepare for transition from the pediatric to the adult health care system. Ideally, transition is a purposeful and coordinated process between the two systems. We sought to evaluate a pediatric rheumatology transition clinic from the perspective of the young adults who attended the clinic. Young adults who attended the IWK Health Centre Pediatric Rheumatology Transition Clinic in Halifax, Nova Scotia, Canada were asked to complete a mail questionnaire. In this clinic an adult rheumatologist joins the pediatric team for the patient’s visit. Subjects rated satisfaction with the clinic and how completely a number of items were addressed (e.g. knowledge about disease, self-management, adolescent issues) on a 10 cm visual analog scale (higher scores reflecting more favourable assessment). Compliance with follow-up post-transfer to adult care was assessed by self-report and a chart review. Data were summarized descriptively. The response rate was 34 % (51/151). The mean age of respondents was 22 years with the majority diagnosed with juvenile idiopathic arthritis. Most patients were transferred to adult care between the ages of 17 and 20 years. The mean overall satisfaction score with the transition clinic was 7.3 ± 2.6. There was significant variability regarding how well individual transition-related items were perceived to have been addressed, with an overall mean of 6.1 ± 3.2. Items which received a majority of scores of > 7 included learning about side effects of medications, learning to live with their disease, confidence in disease management, and control of disease at transfer. Items rated as <5 by a third of respondents included addressing teen issues (smoking, alcohol, sexual health) and learning about new developments related to their condition. 74 % of patients reported regular appointments with adult rheumatology. Most young adults reported overall satisfaction with the transition clinic, however their perception of how adequately various transition issues were addressed was quite variable. It appears that there were some perceived deficits in the care that was provided in all areas, but possibly more so in counselling around general adolescent issues. There was a high rate of follow-up after transfer to the local adult clinic.

Objective: To determine the bone mineral density (BMD) status of our juvenile dermatomyositis (DM) population and to compare the frequency of osteopenia in patients with active disease requiring corticosteroids with that in patients with inactive disease who are not receiving corticosteroids.Methods: Medical charts of all children diagnosed as having juvenile DM at our institution between 1989 and 1999 were reviewed for demographic and clinical data, including disease activity and duration of corticosteroid therapy. BMD measurements of the lumbar spine (L1–L4) were performed using dual x‐ray absorptiometry (DXA). Z scores were calculated from the BMD data for comparison with published normative data.Results: A total of 15 patients were assessed: 10 with active disease, and 5 with inactive disease who had not taken corticosteroids for an average of 6.0 years (range 3.4–8.1 years). Baseline BMD measurements demonstrated osteopenia or frank osteoporosis in the majority of patients, including 6 of the 10 patients with active disease and 4 of the 5 patients whose disease was in remission. Fourteen patients had serial BMD measurements. Persistent or worsening osteopenia was documented in all patients who had ongoing active disease, except for 3 patients who had been treated with bisphosphonates because of vertebral compression fractures.Conclusion: Osteopenia is common in patients with juvenile DM, and it usually worsens with ongoing disease. It can persist for many years after the disease enters remission. Bisphosphonates appeared to beneficially affect bone mineralization in our patients. Treatment to prevent the long‐term complications of osteoporosis in patients with juvenile DM should be considered and requires further study.

Kawasaki Disease (KD), the leading cause of acquired heart disease in children in the developed world, is extremely rare in neonates. We present a case of incomplete KD in a neonate and a review of the literature on neonatal KD. A previously healthy full term 15 day old Caucasian male with an unremarkable antenatal and perinatal history, presented on Day 2 of illness with fever, rash, irritability, and poor feeding. Examination revealed fever (39.6C), tachycardia, tachypnea, extreme irritability, and a generalized maculopapular rash, but was otherwise normal. His complete blood count, CRP and ESR were normal. Empiric intravenous antibiotics and acyclovir resulted in no improvement. On day 4, he had ongoing fever and developed recurrent apnea, required supplemental oxygen, and was transferred to the pediatric intensive care unit. On day 6, he developed bilateral non-purulent conjunctivitis, palmar erythema, bilateral non-pitting edema and erythema of his feet, and arthritis. His full septic work-up and viral studies were negative. On Day 7 he was treated with intravenous immunoglobulin, and over the next 48 h his symptoms including extremity edema resolved, he no longer required supplemental oxygen, and fever did not recur. On day 9 of illness he had marked thrombocytosis. Subsequently, he developed distal extremity desquamation. Repeated echocardiograms excluded the presence of coronary artery aneurysms (CAA). We believe this to be a rare case of incomplete KD in a neonate, in which timely IVIG administration led to resolution of the acute illness and may have prevented CAA. A comprehensive English-language medical literature review of KD presenting in the neonatal period revealed only fifteen case reports. Cases often presented with incomplete KD, and a number had atypical laboratory features including a normal CRP in the acute phase, similar to what was seen in our patient. This case and our literature review should increase awareness that KD can rarely occur in neonates, often presenting atypically. Recognizing KD in a neonate enables appropriate treatment that can result in resolution of symptoms and may decrease the risk of cardiac complications.

The management of background corticosteroid therapy in rheumatology clinical trials poses a major challenge. We describe the consensus methodology used to design an algorithm to standardize changes in corticosteroid dosing during the Randomized Placebo Phase Study of Rilonacept in Systemic Juvenile Idiopathic Arthritis Trial (RAPPORT). The 20 RAPPORT site principal investigators (PIs) and 4 topic specialists constituted an expert panel that participated in the consensus process. The panel used a modified Delphi Method consisting of an on-line questionnaire, followed by a one day face-to-face consensus conference. Consensus was defined as ≥ 75% agreement. For items deemed essential but when consensus on critical values was not achieved, simple majority vote drove the final decision. The panel identified criteria for initiating or increasing corticosteroids. These included the presence or development of anemia, myocarditis, pericarditis, pleuritis, peritonitis, and either complete or incomplete macrophage activation syndrome (MAS). The panel also identified criteria for tapering corticosteroids which included absence of fever for ≥ 3 days in the previous week, absence of poor physical functioning, and seven laboratory criteria. A tapering schedule was also defined. The expert panel established consensus regarding corticosteroid management and an algorithm for steroid dosing that was well accepted and used by RAPPORT investigators. Developed specifically for the RAPPORT trial, further study of the algorithm is needed before recommendation for more general clinical use.