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OBJECTIVES: Using an electronic health record (EHR)-enabled pediatric lupus registry, we evaluated high-quality care delivery in the context of provider goal-setting activities and a multidisciplinary care model. We then determined associations between care quality and prednisone use among youth with systemic lupus erythematosus (SLE).
METHODS: We implemented standardized EHR documentation tools to auto-populate a SLE registry. We compared pediatric lupus care index (p-LuCI) performance (range 0.0-1.0; 1.0 representing perfect metric adherence) and timely follow-up a) before vs. during provider goal-setting activities and population management, and b) in multidisciplinary lupus nephritis vs. rheumatology clinic. We estimated associations between p-LuCI and subsequent prednisone use, adjusted for time, current medication, disease activity, clinical features, and social determinants of health.
RESULTS: We analyzed 830 visits by 110 patients (median 7 visits/patient [IQR 4-10]) over 3.5 years. The provider-directed activity was associated with improved p-LuCI performance (adjusted β 0.05, 95%CI [0.01-0.09]; mean 0.74 vs. 0.69). Patients with nephritis in multidisciplinary clinic had higher p-LuCI (adjusted β 0.06, 95%CI [0.02-0.10]) and likelihood of timely follow-up than those in rheumatology (adjusted RR 1.27, 95%CI [1.02-1.57]). p-LuCI ≥0.50 was associated with 0.72-fold lower adjusted risk of subsequent prednisone use (95%CI [0.53-0.93]). Minoritized race, public insurance, and living in areas with greater social vulnerability were not associated with reduced care quality or follow-up, but public insurance was associated with higher risk of prednisone use.
CONCLUSION: Greater attention to quality metrics associates with better outcomes in childhood SLE. Multidisciplinary care models with population management may additionally facilitate equitable care delivery. This article is protected by copyright. All rights reserved.
OBJECTIVES: Concomitant arthritis may increase risk of chronic opioid use in youngsters with IBD. We aimed to assess trends and clinical features associated with opioid use in children with IBD-related arthritis.
METHODS: Adolescents under 18 years of age with IBD-related arthritis, at least 1 year of continuous enrolment, and at least 1 pharmacy claim in the Truven Health MarketScan Claims and Encounter Database were included. Subjects were identified using previously validated algorithms consisting of ICD codes, pharmacy claims and procedure codes. The primary outcome was chronic opioid exposure. Temporal trends in opioid exposure were tested using the Cuzick-Wilcoxon test. The association of chronic opioid use and baseline covariates in the IBD and IBD-arthritis cohorts were examined using multivariable logistic regression models.
RESULTS: 14,943 adolescents with IBD, 480 of whom had arthritis, were included. Chronic opioid use was non-trivial in youngsters with IBD-related arthritis, higher than that of total IBD cohort (12.3% vs. 5%) and remained stable over the years of study. Using multivariable regression, joint pain and arthritis were significantly associated with chronic opioid exposure in young people with IBD. Among IBD-related arthritis patients older age, public insurance, gastrointestinal surgery, hospitalisation and psychiatric comorbidities were significantly associated with chronic opioid use.
CONCLUSIONS: Chronic opioid use in adolescents with IBD-related arthritis was higher than that of total IBD cohort but stable over the years of study. Future study is needed to explore ways to optimise non-narcotic pain management strategies and ensuring appropriate use of opioids when necessary.
OBJECTIVES: Using an electronic health record (EHR)-enabled pediatric lupus registry, we evaluated high-quality care delivery in the context of provider goal-setting activities and a multidisciplinary care model. We then determined associations between care quality and prednisone use among youth with systemic lupus erythematosus (SLE).
METHODS: We implemented standardized EHR documentation tools to auto-populate a SLE registry. We compared pediatric lupus care index (p-LuCI) performance (range 0.0-1.0; 1.0 representing perfect metric adherence) and timely follow-up a) before vs. during provider goal-setting activities and population management, and b) in multidisciplinary lupus nephritis vs. rheumatology clinic. We estimated associations between p-LuCI and subsequent prednisone use, adjusted for time, current medication, disease activity, clinical features, and social determinants of health.
RESULTS: We analyzed 830 visits by 110 patients (median 7 visits/patient [IQR 4-10]) over 3.5 years. The provider-directed activity was associated with improved p-LuCI performance (adjusted β 0.05, 95%CI [0.01-0.09]; mean 0.74 vs. 0.69). Patients with nephritis in multidisciplinary clinic had higher p-LuCI (adjusted β 0.06, 95%CI [0.02-0.10]) and likelihood of timely follow-up than those in rheumatology (adjusted RR 1.27, 95%CI [1.02-1.57]). p-LuCI ≥0.50 was associated with 0.72-fold lower adjusted risk of subsequent prednisone use (95%CI [0.53-0.93]). Minoritized race, public insurance, and living in areas with greater social vulnerability were not associated with reduced care quality or follow-up, but public insurance was associated with higher risk of prednisone use.
CONCLUSION: Greater attention to quality metrics associates with better outcomes in childhood SLE. Multidisciplinary care models with population management may additionally facilitate equitable care delivery. This article is protected by copyright. All rights reserved.
There is little data to inform use of renin angiotensin aldosterone system (RAAS) inhibitors in pediatric patients with systemic lupus erythematosus (SLE). Here, we sought to characterize RAAS inhibitor use in pediatric SLE and determine whether early RAAS inhibitor initiation among children with incident lupus nephritis is associated with decreased duration of chronic glucocorticoid exposure. A retrospective cohort study was performed of children (ages 5-18) with SLE and/or lupus nephritis in the Truven MarketScan™ Medicaid and Commercial databases (2013-2018) and estimated RAAS inhibitor use. Among incident nephritis cases, we used competing risk hazard models with inverse probability of treatment weighting to estimate the association between RAAS inhibitor initiation less than 180 days after diagnosis and time to glucocorticoid discontinuation with kidney failure as a competing event. Among 592 children with nephritis and 1407 children with non-kidney SLE, 67% and 15% ever received RAAS inhibitors, respectively. Median duration of RAAS inhibitor use among 323 incident users was 14 and 9 months in children with and without nephritis, respectively. Medicaid enrollment was independently associated with greater likelihood of RAAS inhibitor use, irrespective of nephritis. Among 158 incident nephritis cases, early RAAS inhibitor initiation was significantly associated with a faster rate of glucocorticoid discontinuation (adjusted sub-distribution hazard ratio 1.81, 95% confidence interval [1.09 - 3.00]). Thus, early initiation of RAAS inhibitors may have a role in children newly diagnosed with lupus nephritis; not only those with refractory proteinuria after induction therapy. Hence, integrated health systems data could be leveraged to confirm these findings and optimize adjunctive therapies in pediatric lupus.
<p><strong>OBJECTIVES: </strong>This study evaluated the clinical features, treatment patterns, and short-term outcomes of children with inflammatory bowel disease (IBD)-associated musculoskeletal manifestations.</p>
<p><strong>METHODS: </strong>This was a retrospective cohort study of children with IBD evaluated for joint complaints in a paediatric rheumatology clinic from 2015 to 2020. The index visit was the date of initial rheumatology evaluation. Clinical features were evaluated using standard descriptive statistics. Differences in outcomes over time were compared using rank-sum tests. Univariate logistic regression was used to test associations between clinical features and persistent arthritis or enthesitis.</p>
<p><strong>RESULTS: </strong>Seventy-five patients met inclusion criteria. 61% had active arthritis or enthesitis at initial evaluation, 1/3 of whom were not yet diagnosed with IBD. Of those with known IBD, over half with joint complaints had arthritis or enthesitis. Active joint disease was common even among patients already receiving tumour necrosis factor (TNF) inhibitors or other immunomodulatory medications for IBD and despite inactive gastrointestinal disease. Treatment escalation was often needed to control articular disease, which included changes in immunomodulatory therapy and NSAIDs. Treatment outcomes for arthritis were good and significant improvements in functional mobility were observed (p<0.01), while enthesitis often persisted at follow-up (11/28, 39%). Moreover, a significant proportion of patients with pain at the index visit reported persistent pain at follow-up (29/44, 65%).</p>
<p><strong>CONCLUSIONS: </strong>This study provides several findings relevant to the multidisciplinary care of children with IBD, including high rates of active arthritis and enthesitis despite ongoing use of immunomodulatory medications for the management of IBD, responses to treatment, and pain management.</p>
<p><strong>BACKGROUND AND OBJECTIVES: </strong>Performing adequately powered clinical trials in pediatric diseases, such as SLE, is challenging. Improved recruitment strategies are needed for identifying patients.</p>
<p><strong>DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: </strong>Electronic health record algorithms were developed and tested to identify children with SLE both with and without lupus nephritis. We used single-center electronic health record data to develop computable phenotypes composed of diagnosis, medication, procedure, and utilization codes. These were evaluated iteratively against a manually assembled database of patients with SLE. The highest-performing phenotypes were then evaluated across institutions in PEDSnet, a national health care systems network of >6.7 million children. Reviewers blinded to case status used standardized forms to review random samples of cases (=350) and noncases (=350).</p>
<p><strong>RESULTS: </strong>Final algorithms consisted of both utilization and diagnostic criteria. For both, utilization criteria included two or more in-person visits with nephrology or rheumatology and ≥60 days follow-up. SLE diagnostic criteria included absence of neonatal lupus, one or more hydroxychloroquine exposures, and either three or more qualifying diagnosis codes separated by ≥30 days or one or more diagnosis codes and one or more kidney biopsy procedure codes. Sensitivity was 100% (95% confidence interval [95% CI], 99 to 100), specificity was 92% (95% CI, 88 to 94), positive predictive value was 91% (95% CI, 87 to 94), and negative predictive value was 100% (95% CI, 99 to 100). Lupus nephritis diagnostic criteria included either three or more qualifying lupus nephritis diagnosis codes (or SLE codes on the same day as glomerular/kidney codes) separated by ≥30 days or one or more SLE diagnosis codes and one or more kidney biopsy procedure codes. Sensitivity was 90% (95% CI, 85 to 94), specificity was 93% (95% CI, 89 to 97), positive predictive value was 94% (95% CI, 89 to 97), and negative predictive value was 90% (95% CI, 84 to 94). Algorithms identified 1508 children with SLE at PEDSnet institutions (537 with lupus nephritis), 809 of whom were seen in the past 12 months.</p>
<p><strong>CONCLUSIONS: </strong>Electronic health record-based algorithms for SLE and lupus nephritis demonstrated excellent classification accuracy across PEDSnet institutions.</p>
<p><strong>BACKGROUND: </strong>We aimed to test if standardized point-of-care outcome monitoring and clinical decision support (CDS), as compared to standard care, improves disease activity and patient-reported pain in children with enthesitis-related arthritis (ERA).</p>
<p><strong>METHODS: </strong>This was a retrospective cohort study of outcomes of children with ERA after phased implementation of I) standardized outcome monitoring with CDS for polyarticular JIA, and II) CDS for ERA, compared to a pre-intervention group of historical controls. We used multivariable mixed-effects models for repeated measures to test whether implementation phase or other disease characteristics were associated with change over time in disease activity, as measured by the clinical juvenile arthritis disease activity score (cJADAS), and pain.</p>
<p><strong>RESULTS: </strong>One hundred fifty-two ERA patients (41% incident cases) were included with a median age of 14.9 years. Implementation of standardized outcome monitoring or ERA-specific CDS did not result in significant differences in cJADAS or pain over time compared to the pre-intervention cohort. Higher cJADAS at the index visit, pain and more tender entheses were significantly associated with higher cJADAS scores over time (all p < 0.01), while biologic use was associated with lower cJADAS (p = 0.02). Regardless of intervention period, incident ERA cases had a greater rate of cJADAS improvement over time compared to prevalent cases (p < 0.01), but pain persisted over time among both incident and prevalent cases.</p>
<p><strong>CONCLUSIONS: </strong>There was no significant effect of point-of-care outcome monitoring or CDS interventions on disease activity or pain over time in children with ERA in this single center study. Future efforts to improve disease outcomes using standardized outcome monitoring and CDS will need to consider the importance of addressing pain as a target in addition to spondyloarthritis-specific disease activity metrics.</p>
<p><strong>BACKGROUND: </strong>We sought to evaluate racial disparities in disease outcomes among children with polyarticular juvenile idiopathic arthritis (JIA) during a treat-to-target (TTT) intervention with clinical decision support (CDS).</p>
<p><strong>METHODS: </strong>This was a retrospective analysis of a TTT-CDS strategy integrated into clinical practice for children with polyarticular JIA at a single center from 2016 to 2019. The primary outcome was the clinical Juvenile Arthritis Disease Activity Score (cJADAS-10). We used multivariable linear regression to assess racial differences in disease outcomes at the index visit (first visit after implementation). The effect of race on disease outcomes over time was estimated using linear mixed-effects models, stratified by incident or prevalent disease.</p>
<p><strong>RESULTS: </strong>We included 159 children with polyarticular JIA, of which 74, 13 and 13% were white, black, and Asian/other, respectively. cJADAS-10 improved significantly over time for all race categories, while the rates of improvement did not differ by race in incident (p = 0.53) or prevalent cases (p = 0.58). cJADAS-10 over time remained higher among black children compared to white children (β 2.5, p < 0.01 and β 1.2, p = 0.08 for incident and prevalent cases, respectively). Provider attestation to CDS use at ≥50% of encounters was associated with a 3.9 greater reduction in cJADAS-10 among black children compared to white children (p = 0.02).</p>
<p><strong>CONCLUSION: </strong>Despite similar rates of improvement over time by race, disparities in JIA outcomes persisted throughout implementation of a TTT-CDS approach. More consistent CDS use may have a greater benefit among black children and needs to be explored further.</p>
<p><strong>BACKGROUND: </strong>Strain analysis with speckle-tracking echocardiography shows promise as a screening tool for silent myocardial dysfunction in pediatric-onset systemic lupus erythematosus (pSLE). We compared left ventricular (LV) systolic deformation (measured by strain) in children and adolescents with pSLE to controls, and assessed the relationship between strain, disease activity, and other noninvasive measures of cardiovascular health.</p>
<p><strong>METHODS: </strong>Twenty pSLE subjects ages 9-21 underwent comprehensive cardiovascular testing, including 2D speckle-tracking echocardiography, ambulatory blood pressure monitoring (ABPM), peripheral endothelial function testing, pulse wave velocity and analysis, and carotid ultrasound. Longitudinal apical-4 chamber (LS ) and midpoint circumferential strain (CS ) were compared to that of 70 healthy controls using multivariable linear regression. Among pSLE subjects, Pearson correlation coefficients were calculated to evaluate relationships between global longitudinal or circumferential strain and other measures of cardiovascular health.</p>
<p><strong>RESULTS: </strong>Average SLE disease duration was 3.2 years (standard deviation [SD] 2.1). 2/20 pSLE subjects had persistent disease activity, and only one met criteria for hypertension by ABPM. LS was significantly reduced in pSLE subjects compared to controls (mean -18.3 [SD 3.2] vs -21.8% [SD 2.2], P-value <.001). There was no significant difference in CS (-24.8 [SD 3.7] vs -25.7% [SD 3.4], P = .29). Among pSLE subjects, decreased nocturnal blood pressure dipping on ABPM was associated with reduced global circumferential strain (r -0.59, P = .01).</p>
<p><strong>CONCLUSIONS: </strong>Longitudinal myocardial deformation is impaired in pSLE patients despite clinical remission and may represent early myocardial damage. Strain analysis should be considered in addition to standard echocardiographic assessment during follow-up of patients with pSLE.</p>