Objectives Systemic Juvenile Idiopathic Arthritis (sJIA) is characterized by fevers, rash and arthritis, for which IL1 and IL6 inhibitors appear effective. Pulmonary artery hypertension (PAH), interstitial lung disease (ILD) and alveolar proteinosis (AP) have been recently reported in sJIA patients with increased frequency. Our aim was to characterize and compare these cases to a larger cohort of sJIA patients. Methods sJIA patients who developed PAH, ILD and/or AP were identified through an electronic listserv, and their demographic, sJIA and pulmonary disease characteristics, and medication exposure information were collected. These features were compared to a cohort of sJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Results Patients (N=25) were significantly (p<0.05) more likely than the CARRA registry cohort (N=389) to be female, have more systemic features, and to have been exposed to an IL-1 inhibitor, tocilizumab, infliximab, corticosteroids, intravenous immunoglobulin, cyclosporine and cyclophosphamide. Eighty% were diagnosed after 2004. Twenty (80%) patients had MAS during their disease course and 15 (60%) had MAS at pulmonary diagnosis. Sixteen patients had PAH, 5 AP and 7 ILD. Seventeen (68%) patients were taking or recently (≤1 month) discontinued a biologic agent at pulmonary symptom onset; 12 (48%) were taking anti-IL1 therapy (primarily anakinra). Seventeen (68%) patients died at a mean of 8.8 months from pulmonary diagnosis. Conclusions PAH, AP and ILD are under-recognized complications of sJIA which are frequently fatal. These may be the result of severe uncontrolled systemic disease activity, and may be influenced by medication exposure.

SUMMARY BackgroundInfliximab is typically administered intravenously via 2-to 3-h duration infusions. Infusions are time-consuming and costly. Shorter duration infusions are administered at some centres. Limited safety data are available on shorter duration infusions.

Background The use of telemedicine in pediatric rheumatology has been historically low. The current COVID 19 global pandemic has forced a paradigm shift with many centers rapidly adopting virtual visits to conduct care resulting in rapid expansion of use of telemedicine amongst practices. Body This commentary discusses practical tips for physicians including guidance around administrative and governance issues, preparation for telemedicine, involving the multidisciplinary care team, and teaching considerations. We also outline a standard proforma and smart phrases for the electronic health record. A proposed variation of the validated pediatric gait arms legs spine examination (pGALS) called the video pGALS (VpGALS) as a means of conducting virtual pediatric rheumatology physical examination is presented. Conclusion This commentary provides a starting framework for telemedicine use in pediatric rheumatology and further work on validation and acceptability is needed.

Background The 6 competencies defined by the Accreditation Council for Graduate Medical Education provide the framework of assessment for trainees in the US graduate medical education system, but few studies have investigated their impact on remediation. Methods We obtained data via an anonymous online survey of pediatrics residency program directors. For the purposes of the survey, remediation was defined as “any form of additional training, supervision, or assistance above that required for a typical resident.” Respondents were asked to quantify 3 groups of residents: (1) residents requiring remediation; (2) residents whose training was extended for remediation purposes; and (3) residents whose training was terminated owing to issues related to remediation. For each group, the proportion of residents with deficiencies in each of the 6 competencies was calculated. Results In all 3 groups, deficiencies in medical knowledge and patient care were most common; deficiencies in professionalism and communication were moderately common; and deficiencies in systems-based practice and practice-based learning and improvement were least common. Residents whose training was terminated were more likely to have deficiencies in multiple competencies. Conclusion Although medical knowledge and patient care are reported most frequently, deficiencies in any of the 6 competencies can lead to the need for remediation in pediatrics residents. Residents who are terminated are more likely to have deficits in multiple competencies. It will be critical to develop and refine tools to measure achievement in all 6 competencies as the graduate medical education community may be moving further toward individualized training schedules and competency-based, rather than time-based, training.