Childhood Arthritis and Rheumatology Research Alliance Biologic DMARD Consensus Treatment Plans for Refractory Moderately Severe Juvenile Dermatomyositis.

OBJECTIVE: The objective was to develop consensus treatment plans (CTPs) for patients with refractory moderately severe juvenile dermatomyositis (JDM) treated with biologic disease-modifying antirheumatic drugs (bDMARDs). METHODS: The Biologics Workgroup of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Research Committee used case-based surveys, consensus framework, and nominal group technique to produce bDMARD CTPs for patients with refractory moderately severe JDM. RESULTS: Four bDMARD CTPs were proposed: TNF-alpha inhibitor (adalimumab or infliximab), abatacept, rituximab, and tocilizumab. Each CTP has different options for dosing and/or route. Among 76 respondents, consensus was achieved for the proposed CTPs (93% [67/72]) as well as for patient characteristics, assessments, outcome measures, and follow up. By weighted average, respondents indicated that they would most likely use rituximab followed by abatacept, TNF-alpha inhibitor, and tocilizumab. CONCLUSION: CTPs for the use of bDMARDs in refractory moderately severe JDM were developed using consensus methodology. The implementation of the bDMARD CTPs will lay the groundwork for registry-based prospective comparative effectiveness studies.

Perioperative care of the patient with a periodic fever syndrome.

Periodic fever syndromes are autoinflammatory disorders associated with recurrent fevers unrelated to infection. Little is known about the perioperative management of patients with these syndromes, and existing literature consists primarily of case reports and occasional case series. This narrative review discusses background information and diagnostic criteria for the three most common periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA), familial Mediterranean fever (FMF), and TNF receptor-associated periodic syndrome (TRAPS), and describes perioperative considerations for anesthesia providers when caring for the patient with a periodic fever syndrome. We include a systems-based framework in which to organize these considerations.

Social determinants of health influence disease activity and functional disability in Polyarticular Juvenile Idiopathic Arthritis.

BACKGROUND: Social determinants of health (SDH) greatly influence outcomes during the first year of treatment in rheumatoid arthritis, a disease similar to polyarticular juvenile idiopathic arthritis (pJIA). We investigated the correlation of community poverty level and other SDH with the persistence of moderate to severe disease activity and functional disability over the first year of treatment in pJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry. METHODS: In this cohort study, unadjusted and adjusted generalized linear mixed effects models analyzed the effect of community poverty and other SDH on disease activity, using the clinical Juvenile Arthritis Disease Activity Score-10, and disability, using the Child Health Assessment Questionnaire, measured at baseline, 6, and 12 months. RESULTS: One thousand six hundred eighty-four patients were identified. High community poverty (≥20% living below the federal poverty level) was associated with increased odds of functional disability (OR 1.82, 95% CI 1.28-2.60) but was not statistically significant after adjustment (aOR 1.23, 95% CI 0.81-1.86) and was not associated with increased disease activity. Non-white race/ethnicity was associated with higher disease activity (aOR 2.48, 95% CI: 1.41-4.36). Lower self-reported household income was associated with higher disease activity and persistent functional disability. Public insurance (aOR 1.56, 95% CI 1.06-2.29) and low family education (aOR 1.89, 95% CI 1.14-3.12) was associated with persistent functional disability. CONCLUSION: High community poverty level was associated with persistent functional disability in unadjusted analysis but not with persistent moderate to high disease activity. Race/ethnicity and other SDH were associated with persistent disease activity and functional disability.

Synovectomy in juvenile idiopathic arthritis: A systematic review and meta-analysis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is an inflammatory arthropathy with onset in children younger than 16 years. Treatment is primarily medical; however, surgical interventions, such as arthroscopic or open synovectomy, can be beneficial. Many studies have investigated synovectomy in JIA, but the results of these studies have not been synthesized to our knowledge. Therefore, we performed a systematic review of the literature reporting synovectomy as a treatment for JIA to provide clinical recommendations regarding its risks and benefits. METHODS: On March 8, 2022, we searched the Cochrane Library, Embase, PubMed, Scopus, and Web of Science for studies evaluating clinical outcomes of open or arthroscopic synovectomy to treat JIA in patients younger than 18 years. We included only studies published in English and excluded studies of synovectomy to treat other arthropathies, septic arthritis, hemophilia, or foreign body arthropathy. The level of evidence for included studies was determined by using the Oxford Centre for Evidence-Based Medicine criteria. We qualitatively analyzed clinical outcomes data, including patient-reported pain relief, rates of symptom recurrence, and postoperative complications. RESULTS: Of 428 articles assessed, 14 were included in our analysis. One was a randomized trial, 1 was a case-control study, and all others were case-series. Studies consistently reported that synovectomy was associated with improved function and decreased pain postoperatively. However, comparisons with modern medical therapy were lacking. Rates of arthritis recurrence varied, with increasing symptom recurrence with longer follow-up and re-synovectomy rates up to 15%. Oligoarticular disease and early disease course were associated with better response to synovectomy, whereas systemic and polyarticular disease were associated with poor response. Stiffness requiring manipulation under anesthesia was the most common complication (4% of all included patients). CONCLUSION: Although synovectomy is associated with positive functional outcomes and pain reduction postoperatively, there was inadequate comparison thus inadequate evidence to recommend it over modern medical therapy. The current literature suggests that synovectomy should be offered only to patients for whom medical management has failed, while noting the risks of decreased range of motion and symptom recurrence over time.

Community poverty level influences time to first pediatric rheumatology appointment in Polyarticular Juvenile Idiopathic Arthritis.

BACKGROUND: The impact of social determinants of health on children with polyarticular juvenile idiopathic arthritis (pJIA) is poorly understood. Prompt initiation of treatment for pJIA is important to prevent disease morbidity; however, a potential barrier to early treatment of pJIAs is delayed presentation to a pediatric rheumatologist. We examined the impact of community poverty level, a key social determinant of health, on time from patient reported symptom onset to first pediatric rheumatology visit among pJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. METHODS: This is a cohort study of pJIA patients in the CARRA registry who lived in the United States from July 2015-February 2020. The primary exposure was community poverty level derived by geocoding patient addresses. The primary outcome was time to first rheumatology appointment. Kaplan-Meier analysis was performed to analyze time to first rheumatologist visit, stratified by community poverty and family income. Log-rank tests were used to identify differences between groups. Adjusted cox proportional-hazards models were used to determine the relationship between community poverty level and time from onset of disease symptoms to date first seen by rheumatologist. RESULTS: A total of 1684 patients with pJIA meeting study inclusion and exclusion criteria were identified. Median age of onset of pJIA was 7 years (IQR 3, 11), 79% were female, 17.6% identified as minority race and/or ethnicity, and 19% were from communities with ≥20% community poverty level. Kaplan-Meier analysis by community poverty level (< 20% vs ≥20%) yielded no significant differences with time to initial presentation to a pediatric rheumatologist (p = 0.6). The Cox proportional hazards model showed that patients with ≥20% community poverty level were 19% less likely (adjusted HR 0.81, 95% CI 0.67-0.99, p = 0.038) to be seen by a rheumatologist compared to patients with < 20% community poverty level, at the same time point, after adjusting for sex, race/ethnicity, insurance, education level, morning stiffness, RF status, and baseline CHAQ. CONCLUSION: In this study of pJIA patients in the CARRA registry, increased community poverty level is associated with longer time to presentation to a pediatric rheumatologist after symptom onset.

Glitches in the utilization of telehealth in pediatric rheumatology patients during the COVID-19 pandemic.

Telehealth is an extraordinary advancement of modern medicine. It has increased access to care for underserved populations and, in the case of pediatric rheumatology, has expanded the reach of a limited work force. During the Coronavirus Disease 2019 (COVID-19) pandemic, telehealth has radically changed the way healthcare workers have been able to deliver care while maintaining social distance. In addition to the infectious havoc of COVID-19, the pandemic has further altered the psychosocial milleu of our society which directly impacts the wellness and safety of our pediatric rheumatology patients. These psychosocial factors may be difficult to assess and triage solely using telehealth. The objective of this short review is to educate practitioners on the psychosocial concerns exacerbated by the COVID-19 pandemic and to discuss the possible hurdles in utilization of telehealth to care for our vulnerable patient population.