Implementation of Rheumatology Health Care Transition Processes and Adaptations to Systems Under Stress: A Mixed-Methods Study.

OBJECTIVES: Despite poor health care transition outcomes among young adults with pediatric rheumatic diseases, adoption of transition best practices is low. We sought to understand how structured transition processes were operationalized within pediatric rheumatology practices and what factors were perceived to enable adaptations during a global pandemic. METHODS: We conducted a mixed methods study of team leaders' experiences during an interim analysis of a pilot project to implement transition policy discussions at sites in the Childhood Arthritis and Rheumatology Research Alliance Transition Learning Collaborative. We combined quantitative assessments of organizational readiness for change (9 sites) and semistructured interviews of team leaders (8 sites) using determinants in the Exploration, Preparation, Implementation, Sustainment Framework. RESULTS: Engagement of nursing and institutional improvement efforts facilitated decisions to implement transition policies. Workflows incorporating educational processes by nonphysicians were perceived to be critical for success. When the pandemic disrupted contact with nonphysicians, capacity for automation using electronic medical record (EMR)-based tools was an important facilitator, but few sites could access these tools. Sites without EMR-based tools did not progress despite reporting high organizational readiness to implement change at the clinic level. Lastly, educational processes were often superseded by acute issues, such that youth with greater medical/psychosocial complexity may not receive the intervention. CONCLUSION: We generated several considerations to guide implementation of transition processes within pediatric rheumatology from the perspectives of team leaders. Careful assessment of institutional and nursing support is advisable before conducting complex transition interventions. Ideally, new strategies would ensure interventions reach youth with high complexity.

Pediatric to Adult Transition Literature: Scoping Review and Rheumatology Research Prioritization Survey Results.

The transition from pediatric to adult care is the focus of growing research. It is important to identify how to direct future research efforts for maximum effect. Our goals were to perform a scoping review of the transition literature, highlight gaps in transition research, and offer stakeholder guidance on the importance and feasibility of research questions designed to fill identified gaps. The transition literature on rheumatic diseases and other common pediatric-onset chronic diseases was grouped and summarized. Based on the findings, a survey was developed and disseminated to pediatric rheumatologists and young adults with rheumatic diseases as well as their caregivers. The transitional care needs of patients, healthcare teams, and caregivers is well described in the literature. While various transition readiness scales exist, no longitudinal posttransfer study confirms their predictive validity. Multiple outcome measures are used alone or in combination to define a successful transition or intervention. Multimodal interventions are most effective at improving transition-related outcomes. How broader health policy affects transition is poorly studied. Research questions that ranked highest for importance and feasibility included those related to identifying and tracking persons with psychosocial vulnerabilities or other risk factors for poor outcomes. Interventions surrounding improving self-efficacy and health literacy were also ranked highly. In contrast to healthcare teams (n = 107), young adults/caregivers (n = 23) prioritized research surrounding improved work, school, or social function. The relevant transition literature is summarized and future research questions prioritized, including the creation of processes to identify and support young adults vulnerable to poor outcomes.

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Therapies, Medication Monitoring, Immunizations, and Imaging.

OBJECTIVE: To provide recommendations for the management of juvenile idiopathic arthritis (JIA) with a focus on nonpharmacologic therapies, medication monitoring, immunizations, and imaging, irrespective of JIA phenotype. 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: Recommendations in this guideline include the use of physical therapy and occupational therapy interventions; a healthy, well-balanced, age-appropriate diet; specific laboratory monitoring for medications; widespread use of immunizations; and shared decision-making with patients/caregivers. Disease management for all patients with JIA is addressed with respect to nonpharmacologic therapies, medication monitoring, immunizations, and imaging. 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, and a concurrent 2021 guideline on oligoarthritis, temporomandibular arthritis, and systemic JIA. 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.

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.

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.

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Therapies, Medication Monitoring, Immunizations, and Imaging.

OBJECTIVE: To provide recommendations for the management of juvenile idiopathic arthritis (JIA) with a focus on nonpharmacologic therapies, medication monitoring, immunizations, and imaging, irrespective of JIA phenotype. 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: Recommendations in this guideline include the use of physical therapy and occupational therapy interventions; a healthy, well-balanced, age-appropriate diet; specific laboratory monitoring for medications; widespread use of immunizations; and shared decision-making with patients/caregivers. Disease management for all patients with JIA is addressed with respect to nonpharmacologic therapies, medication monitoring, immunizations, and imaging. 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, and a concurrent 2021 guideline on oligoarthritis, temporomandibular arthritis, and systemic JIA. 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.

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.

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.

Pediatric BehÒ«et disease: Update in diagnosis and management.

BehÒ«et disease is a multisystem inflammatory disease and variable vessel vasculitis involving primarily the oral and genital mucosa, skin, and eyes. Diagnosis is challenging due to the lack of a specific diagnostic test and overlap with other autoinflammatory diseases. Treatment of pediatric BehÒ«et disease aims to reduce inflammation and prevent future flares. The goal of this review is to provide guidance on the diagnostic workup and multidisciplinary approach of pediatric BehÒ«et disease and review evidence-based treatment strategies for patients with refractory mucocutaneous manifestations.

A case of prolidase deficiency in a male patient.

Prolidase deficiency is an extremely rare, autosomal recessive disorder resulting in defective collagen formation. We report a case of prolidase deficiency in a male child, highlighting the dermatologic features. Early diagnosis is important as these patients encounter significant multisystem comorbidities requiring multispecialty care.

Changes over time in inflammatory and structural lesions at the sacroiliac joint in children with spondyloarthritis exposed and unexposed to tumor necrosis factor inhibitor.

BACKGROUND: The objective of this work was to describe magnetic resonance imaging (MRI) changes over time in inflammatory and structural lesions at the sacroiliac joint (SIJ) in children with spondyloarthritis (SpA) exposed and unexposed to tumor necrosis factor inhibitor (TNFi). METHODS: This was a retrospective, multicenter study of SpA patients with suspected or confirmed sacroiliitis who underwent at ≥2 pelvic MRI scans. Images were reviewed independently by 3 radiologists and scored for inflammatory and structural changes using the Spondyloarthritis Research Consortium of Canada (SPARCC) SIJ inflammation score (SIS) and structural score (SSS). Longitudinal, quantitative changes in patient MRI scans were measured using descriptive statistics and stratified by TNFi exposure. We used an average treatment effects (ATE) regression model to explore the average effect of TNFi exposure over time on inflammatory and structural lesions, adjusting for baseline lesion scores. RESULTS: Forty-six subjects were evaluated using the SIS (n = 45) and SSS (n = 18). Median age at baseline imaging was 13.6 years, 63% were male and 71% were white. Twenty-three subjects (50%) were TNFi exposed between MRI studies. The median change in SIS in TNFi exposed and unexposed subjects with a baseline SIS ≥0 was - 20.7 and - 14.3, respectively (p = 0.09). Eleven (85%) TNFi exposed and 8 (89%) unexposed subjects with a baseline SIS ≥0 met the SIS minimal clinically important difference (MCID; ≥2.5). Using the ATE model adjusted for baseline SIS, the average effect of TNFi on SIS in patients with a baseline SIS ≥2 was - 14.5 (p < 0.01). Unadjusted erosion change score was significantly worse in TNFi unexposed versus exposed subjects (p = 0.03) but in the ATE model the effect of TNFi was not significant. CONCLUSION: This study quantitatively describes how lesions in the SIJs on MRI change over time in patients exposed to TNFi versus unexposed. Follow-up imaging in TNFi exposed patients showed greater improvement than the unexposed group by most metrics, some of which reached statistical significance. Surprisingly, a majority of TNFi unexposed children with a baseline SIS≥2 met the SIS MCID. Additional studies assessing the short and long-term effects of TNFi on inflammatory and structural changes in juvenile SpA are needed.

Factors Associated With Variation in Pediatric Systemic Lupus Erythematosus Care Delivery.

OBJECTIVE: Patients with pediatric systemic lupus erythematosus (pSLE) and mixed connective tissue disease (MCTD) receive only a fraction of recommended care. Using published quality indicators and guidelines, we developed a 13-item pediatric lupus care index (p-LuCI) to quantify the proportion of recommended clinical evaluations and comorbidity prevention interventions completed and the timeliness of follow-up. Our objective was to assess baseline index performance and identify sources of p-LuCI variation. METHODS: We performed a cross-sectional study in patients with pSLE or MCTD and analyzed the performance of individual p-LuCI process metrics and calculated the overall p-LuCI score. We identified factors associated with the p-LuCI using multivariable linear regression with clustering by provider. RESULTS: For 110 patients (99 with pSLE and 11 with MCTD), the median p-LuCI was 65.2% (interquartile range: 9.1-92.3%). Component performance ranged from 27.3% (on-time scheduling) to 95.4% (steroid-sparing treatment). Patients with p-LuCI scores above the median had higher scores across all 13 components. Higher p-LuCI scores were independently associated with disease-modifying antirheumatic drug use (ß = 14.3 [95% confidence interval (CI), 1.5-27.2]), nephritis (ß = 10.4 [95% CI, 5.1-15.8]), higher provider pSLE/MCTD volume (ß = 3.1 [95% CI, 1.9-4.2] per patient), assignment to rheumatology fellow trainee (ß = 36.3 [95% CI, 17.3-55.2]), and disease duration of less than 1 year (ß = 12.6 [95% CI, 0.7-24.5]). Differences by race, ethnicity, and/or insurance were not observed. CONCLUSION: Using an index of recommended pSLE care metrics, we identified significant variation in performance by disease, treatment, and provider characteristics. The p-LuCI may be useful to assess care quality at the patient, provider, and practice levels and to identify areas in need of greater standardization.

Effect of first-line biologic initiation on glucocorticoid exposure in children hospitalized with new-onset systemic juvenile idiopathic arthritis: emulation of a pragmatic trial using observational data.

BACKGROUND: Glucocorticoid exposure is a significant driver of morbidity in children with systemic juvenile idiopathic arthritis (sJIA). We determined the effect of early initiation of biologic therapy (IL-1 or IL-6 inhibition) on glucocorticoid exposure in hospitalized patients with new-onset sJIA. METHODS: We emulated a pragmatic sequence of trials ("pseudo-trials") of biologic initiation in children (≤ 18 years) hospitalized with new-onset sJIA utilizing retrospective data from an administrative database from 52 tertiary care children's hospitals from 2008 to 2019. Eligibility window, treatment assignment and start of follow-up between biologic and non-biologic study arms were aligned for each pseudo-trial. Patients in the source population could meet eligibility criteria at several timepoints. Mixed-effects logistic regression was used to determine the effect of biologic initiation on in-hospital glucocorticoid exposure. RESULTS: Four hundred sixty-eight children met eligibility criteria, of which 19% received biologic therapy without preceding or concomitant initiation of immunomodulatory medications. This proportion significantly increased over time during the study period (p <  0.01). 1451 trial subjects were included across 4 pseudo-trials with 71 assigned to the biologic arm and 1380 assigned to the non-biologic arm. After adjustment, there was a trend toward decreased odds of glucocorticoid initiation in the biologic arm compared to the non-biologic arm (OR 0.39, 95% CI [0.13, 1.15]). CONCLUSION: Biologic initiation in children hospitalized with new-onset sJIA significantly increased over time and may be associated with reduced glucocorticoid exposure. The increasing use of first-line biologic therapy may lead to clinically relevant reductions in treatment-related adverse effects of glucocorticoid-reliant therapeutic approaches.

Variation in Treatment of Children Hospitalized With New-Onset Systemic Juvenile Idiopathic Arthritis in the US.

OBJECTIVE: Increasing evidence supports the conclusion that early initiation of biologics may dramatically improve disease course and reduce glucocorticoid exposure for children with systemic juvenile idiopathic arthritis (JIA). The present study was undertaken to characterize variation in the use of first-line biologic and glucocorticoid therapy and to identify drivers of variation in children hospitalized with new-onset systemic JIA. METHODS: We conducted a retrospective cohort study of children hospitalized with new-onset systemic JIA from 2008 to 2019 utilizing a comparative pediatric database from 52 tertiary care children's hospitals. Subjects and treatment receipt were identified using International Classification of Diseases, Ninth Revision (ICD-9) and ICD-10 discharge diagnosis codes, pharmacy billing data, and clinical transaction classification codes. Mixed-effects logistic regression was used to identify patient- and hospital-level factors associated with receipt of glucocorticoids and biologics. RESULTS: In total, 534 children with new-onset systemic JIA hospitalized during the study period met inclusion criteria. Twenty-nine percent received biologics, and 58% received glucocorticoids. Biologic use increased over time (P < 0.001), methotrexate use decreased (P < 0.01), and glucocorticoid use remained unchanged. Biologics and glucocorticoid use varied significantly between hospitals. High annual hospital volume, intensive care unit stay, and later discharge year were significantly associated with biologic exposure. Medium-high and high annual hospital volume were significantly associated with less glucocorticoid exposure. CONCLUSION: Despite increasing evidence demonstrating improved outcomes with first-line treatment with biologics, we found significant treatment variation across hospitals with many children not receiving biologics and a persistent high rate of glucocorticoid exposure. These results underscore the need for comparative efficacy studies and improved treatment standardization.

Enhancing communication and social engagement among clinicians and research teams to improve reliability of research recruitment.

The success of rare disease research relies heavily on robust partnerships with clinicians to help identify new patients and collect samples. Many studies for paediatric rheumatic diseases requiring pretreatment samples have suffered from slow enrolment rates due to the low incidence of disease and relative urgency to treat. Therefore, timely identification of all potentially eligible patients is crucial. The objective of this project was to apply quality improvement methods to increase the frequency and timeliness of identification of eligible patients with new paediatric rheumatic diagnoses to approach for research studies. A retrospective chart review was undertaken in our paediatric rheumatology clinic to measure the number of eligible patients identified for potential research recruitment between missed recruitment opportunities. Improvement methodology was used to integrate standardised communication between clinicians and the research team into clinic workflow, to leverage social feedback as positive reinforcement for good communication and to measure change in response to the interventions. The number of eligible patients identified between missed recruitment opportunities increased from every 0-1 patient to every 14 patients during the project period, corresponding to an increase in the overall identification rate from 32% to 91% of all eligible patients. Quality improvement methods can be used to successfully integrate research recruitment into routine clinical care and accelerate advances necessary to improve health outcomes.

Rapid high-volume population screening for three major risk factors of future stroke: phase I results.

Three proximate risk factors for stroke are carotid stenosis, atrial fibrillation, and hypertension. Phase I of this prospective study was designed to establish the prevalence of these conditions among a population of health maintenance organization beneficiaries by using a rapid screening protocol in order to risk-stratify patients for appropriate management and subsequent cohort analysis. Patients at a tertiary care medical center were screened for stroke risk by using directed history, a 3-minute carotid "quick-scan'' protocol, an EKG lead II rhythm strip, and bilateral arm blood pressures. Patients with any abnormal result underwent specific diagnostic consultation with vascular surgery, cardiology, or primary care. These evaluations included formal carotid duplex ultrasound, 12-lead EKG +/- Holter monitor, and 5-day blood pressure check. Patients were then stratified into risk cohorts for appropriate management and future analysis of stroke incidence and outcomes. In 8 hours on a single day in October 2002, 294 patients (mean age 69) were screened. Combining history with results of screening and diagnostic tests, the overall prevalence of carotid stenosis was 6% (n = 17/294), atrial fibrillation 7% (n = 21/294), and severe hypertension 5% (n = 16/294). Fifty-nine patients (20%) screened positive for carotid stenosis by "quick-scan,'' and 29% (n = 17/59) of these had confirmed stenosis (>50%) in 1 or both arteries by formal duplex. The prevalence of confirmed carotid stenosis was 37% among those screening positive for 1 artery (odds ratio [OR] 14.6; p<0.001) and 75% among those screening positive for both (OR 74.7; p<0.001). Significant independent predictors of carotid stenosis by multivariate analysis included coronary artery disease or myocardial infarction, smoking, stroke or transient ischemic attack, male gender, and white race (all p<0.05). The prevalence of confirmed stenosis was 10% with any 3 predictors alone (OR 2.5; p<0.05), 31% with any 4 (OR 21.2; p<0.001), and 50% with all 5 (OR 46.5; p<0.001). Thirty-three patients (11%) were found to have a previously unidentified and untreated arrhythmia, and 12% (n = 4/33) of these had confirmed new atrial fibrillation; 158 patients (54%) had moderate hypertension and 16 (5%) had severe hypertension (>180/100). Overall, 82% (n = 242/294) of patients screened required additional diagnostic tests. Based on these results, 11% (n = 31/294) of patients were stratified as high risk, 64% (n = 188/294) as moderate risk, and 25% (n = 75/294) as low risk for future stroke. Rapid and efficient screening of a large population for stroke risk factors is feasible. The prevalence of undiagnosed, unsurveilled, and untreated carotid stenosis, atrial fibrillation, and severe hypertension is significant, as 75% of patients screened had 1 or more confirmed major risk factors for stroke. Phase II of this study will investigate the degree of stroke risk reduction possible with a multidisciplinary approach to early identification and aggressive treatment of these risks.