Objective To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided. Methods We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations. Results Similar to those published in 2019, these JIA recommendations are based on clinical phenotypes of JIA, rather than a specific classification schema. This guideline provides recommendations for initial and subsequent treatment of JIA with oligoarthritis, TMJ arthritis, and systemic JIA as well as for tapering and discontinuing treatment in subjects with inactive systemic JIA. Other aspects of disease management, including factors that influence treatment choice and medication tapering, are discussed. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional. Conclusion This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis. It serves as a tool to support clinicians, patients, and caregivers in decision‐making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision‐making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver.
Objective Systemic juvenile idiopathic arthritis–associated lung disease (SJIA‐LD) is a life‐threatening disease complication. Key questions remain regarding clinical course and optimal treatment approaches. The objectives of the study were to detail management strategies after SJIA‐LD detection, characterize overall disease courses, and measure long‐term outcomes. Methods This was a prospective cohort study. Clinical data were abstracted from the electronic medical record, including current clinical status and changes since diagnosis. Serum biomarkers were determined and correlated with presence of LD. Results We enrolled 41 patients with SJIA‐LD, 85% with at least one episode of macrophage activation syndrome and 41% with adverse reactions to a biologic. Although 93% of patients were alive at last follow‐up (median 2.9 years), 37% progressed to requiring chronic oxygen or other ventilator support, and 65% of patients had abnormal overnight oximetry studies, which changed over time. Eighty‐four percent of patients carried the HLA‐DRB1*15 haplotype, significantly more than patients without LD. Patients with SJIA‐LD also showed markedly elevated serum interleukin‐18 (IL‐18), variable C‐X‐C motif chemokine ligand 9 (CXCL9), and significantly elevated matrix metalloproteinase 7. Treatment strategies showed variable use of anti–IL‐1/6 biologics and addition of other immunomodulatory treatments and lung‐directed therapies. We found a broad range of current clinical status independent of time from diagnosis or continued biologic treatment. Multidomain measures of change showed imaging features were the least likely to improve with time. Conclusion Patients with SJIA‐LD had highly varied courses, with lower mortality than previously reported but frequent hypoxia and requirement for respiratory support. Treatment strategies were highly varied, highlighting an urgent need for focused clinical trials.
Abstract Objective To examine the safety and efficacy of the interleukin‐1 (IL‐1) receptor antagonist anakinra as first‐line therapy for systemic juvenile idiopathic arthritis (JIA). Methods Patients with systemic JIA receiving anakinra as part of initial disease‐modifying antirheumatic drug (DMARD) therapy were identified from 11 centers in 4 countries. Medical records were abstracted using a standardized instrument, and resulting data were analyzed to characterize concomitant therapies, clinical course, adverse events, and predictors of outcome. Results Among 46 patients meeting inclusion criteria, anakinra monotherapy was used in 10 patients (22%), while 67% received corticosteroids and 33% received additional DMARDs. Outcomes were evaluated at a median followup interval of 14.5 months. Fever and rash resolved within 1 month in >95% of patients, while C‐reactive protein and ferritin normalized within this interval in >80% of patients. Active arthritis persisted at 1 month in 39% of patients, at 3 months in 27%, and at >6 months of followup in 11%. Approximately 60% of patients, including 8 of 10 receiving anakinra monotherapy, attained a complete response without escalation of therapy. Disease characteristics and treatment were similar in partial and complete responders, except that partial responders were markedly younger at onset (median age 5.2 years versus 10.2 years; P = 0.004). Associated adverse events included documented bacterial infection in 2 patients and hepatitis in 1 patient. Tachyphylaxis was not observed. Conclusion Anakinra as first‐line therapy for systemic JIA was associated with rapid resolution of systemic symptoms and prevention of refractory arthritis in almost 90% of patients during the interval examined. These results justify further study of IL‐1 inhibition as first‐line, rather than rescue, therapy in systemic JIA.
Cardiovascular associations of Crohn’s Disease (CD) are rare and include valvular heart disease, aortitis and aneurysm. We report an 11 year old with thoracic aortitis and aneurysm found during initial presentation of CD. An 11 year old African American female presented with symptoms concerning for inflammatory bowel disease. Work up revealed leukocytosis, and serum evidence of inflammation. Imaging showed thickening of the distal ileum, suggestive of CD. Surprisingly an aneurysm involving the descending thoracic aorta, with wall thickening, concerning for an impending rupture was also found which was confirmed on an angiogram. Subsequent endoscopy revealed ileocolitis; with no evidence of vasculitis. She was started on infliximab and methotrexate. An elective repair with reconstruction of the aneurysm was performed. The resected aorta revealed neutrophilic infiltrate consistent with inflammation. Work up for primary vasculitis was unrevealing. Her symptoms improved and laboratory markers on the current therapy. Prevalence of EIMs in CD ranges from 10% to 50%. Cardiovascular associations in CD are rare. Adult patients with long standing history of CD may rarely develop aortitis, however, presence of aortitis at diagnosis of CD is not yet reported. Noninfectious aortitis can be managed with steroids, immunosuppressants or surgery. Given the findings of the aneurysm, we performed an elective surgical repair. We chose to avoid steroids because of limited complaints related to her disease and to limit her risk of infection during her upcoming surgery. • IBD and aortitis can present simultaneously, especially CD. • Concomitance of IBD and aortitis is most likely to be related, than a mere coincidence. • Aortitis with limited symptoms can be managed with infliximab and methotrexate without the use of high dose steroids.
The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a North American learning health network focused on improving outcomes of children with juvenile idiopathic arthritis (JIA). JIA is a chronic autoimmune disease that can lead to morbidity related to persistent joint and ocular inflammation. PR-COIN has a shared patient registry that tracks twenty quality measures including ten outcome measures of which six are related to disease activity. The network's global aim, set in 2021, was to increase the percent of patients with oligoarticular or polyarticular JIA that had an inactive or low disease activity state from 76% to 80% by the end of 2023.
Objective This report describes our experience in electronic health record (EHR) note modification and creation of an external dashboard to create a local learning health system that contributes to quality improvement and patient care within our pediatric rheumatology clinic. Methods We applied quality improvement methodology to develop a more reliable and accurate system to identify patients with juvenile idiopathic arthritis and track important measures that aide in improving patient care and performance outcomes. From 2019 to 2021, we iteratively modified our outpatient clinic EHR note to include structured data elements to improve longitudinal monitoring. We then validated data transferred to an electronic dashboard external to the EHR and demonstrated utility for identifying an accurate patient population and tracking quality improvement initiatives. Results Creation of the structured data elements improved the identification of patients with JIA with >99% accuracy and without requiring manual review of the chart. Using the dashboard to monitor performance, we improved documentation of critical disease activity measures that resulted in improvement in those scores across the local population of patients with JIA. The structured data elements also enabled us to automate electronic data transfer to a multicenter learning network registry. Conclusion The structured data element modifications made to our outpatient EHR note populate a local dashboard that allows real time access to critical information for patient care, population management, and improvement in quality metrics. The collection and monitoring of structured data can be scaled to other quality improvement initiatives in our clinic and shared with other centers.
Rates of incident treatment were quantified in this study for diabetes mellitus, hypertension, and venous thromboembolism (VTE) associated with oral glucocorticoid exposure in children aged 1-18 years. The retrospective cohort included more than 930,000 children diagnosed with autoimmune diseases (namely, inflammatory bowel disease, juvenile idiopathic arthritis, or psoriasis) or a nonimmune comparator condition (attention-deficit/hyperactivity disorder) identified using US Medicaid claims (2000-2010). Associations of glucocorticoid dose per age- and sex-imputed weight with incident treated diabetes, hypertension, and VTE were estimated using Cox regression models. Crude rates were lowest for VTE (unexposed: 0.5/million person-days (95% confidence interval (CI): 0.4, 0.6); currently exposed: 15.6/million person-days (95% CI: 11.8, 20.1)) and highest for hypertension (unexposed: 6.7/million person-days (95% CI: 6.5, 7.0); currently exposed: 74.4/million person-days (95% CI: 65.7, 83.9)). Absolute rates for all outcomes were higher in unexposed and exposed children with autoimmune diseases compared with those with attention-deficit/hyperactivity disorder. Strong dose-dependent relationships were found between current glucocorticoid exposure and all outcomes (adjusted hazard ratios for high-dose glucocorticoids: for diabetes mellitus, 5.93 (95% CI: 3.94, 8.91); for hypertension, 19.13 (95% CI: 15.43, 23.73); for VTE, 16.16 (95% CI: 8.94, 29.22)). These results suggest strong relative risks, but low absolute risks, of newly treated VTE, diabetes, and especially hypertension in children taking high-dose oral glucocorticoids.
To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided.We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations.Similar to those published in 2019, these JIA recommendations are based on clinical phenotypes of JIA, rather than a specific classification schema. This guideline provides recommendations for initial and subsequent treatment of JIA with oligoarthritis, TMJ arthritis, and systemic JIA as well as for tapering and discontinuing treatment in subjects with inactive systemic JIA. Other aspects of disease management, including factors that influence treatment choice and medication tapering, are discussed. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional.This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis. It serves as a tool to support clinicians, patients, and caregivers in decision-making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision-making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver.
The aim of this study was to describe the adult rheumatology workforce in the United States, assess change in rheumatology providers over time, and identify variation in rheumatology practice characteristics.
