<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&nbsp;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 &lt;.001). There was no significant difference in CS (-24.8 [SD 3.7] vs -25.7% [SD 3.4], P&nbsp;=&nbsp;.29). Among pSLE subjects, decreased nocturnal blood pressure dipping on ABPM was associated with reduced global circumferential strain (r -0.59, P&nbsp;=&nbsp;.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>

<p><strong>OBJECTIVE: </strong>Youth with systemic lupus erythematosus (SLE) experience high rates of psychiatric comorbidities, which may affect medication adherence. We examined the association between psychiatric disorders and hydroxychloroquine adherence and determined whether psychiatric treatment modifies this association.</p>

<p><strong>METHODS: </strong>We identified incident hydroxychloroquine users among youth with SLE (ages 10-24 years) using de-identified U.S. commercial insurance claims in Optum Clinformatics® Data Mart (2000-2016). Adherence was estimated using medication possession ratios (MPR) over a 365-day interval. Multivariable linear regression models were used to estimate the effect of having any psychiatric disorder on MPR, as well as the independent effects of depression, anxiety, adjustment and other psychiatric disorders. We tested for interactions between psychiatric diagnoses and treatment with psychotropic medications or psychotherapy.</p>

<p><strong>RESULTS: </strong>Among 873 subjects, 20% had a psychiatric diagnosis, most commonly depression. Only adjustment disorders were independently associated with decreased MPRs (β -0.12, p=0.05). We observed significant crossover interactions, in which psychiatric disorders had opposite effects on adherence depending on the receipt of psychiatric treatment. Among youth with any psychiatric diagnosis, psychotropic medication use was associated with a 0.15 increase in MPR compared with no psychotropic medication (p=0.02 for interaction). Among youth with depression or anxiety, psychotherapy was also associated with a higher MPR compared with no psychotherapy (p=0.05 and p&lt;0.01 for interaction, respectively).</p>

<p><strong>CONCLUSION: </strong>The impact of psychiatric disorders on medication adherence differed by whether youth had received psychiatric treatment. Improving recognition and treatment of psychiatric conditions may increase adherence in youth with SLE.</p>

<p><strong>BACKGROUND: </strong>Loss of the normal nocturnal decline in blood pressure (BP), known as non-dipping, is a potential measure of cardiovascular risk identified by ambulatory blood pressure monitoring (ABPM). We sought to determine whether non-dipping is a useful marker of abnormal vascular function and subclinical atherosclerosis in pediatric-onset systemic lupus erythematosus (pSLE).</p>

<p><strong>METHODS: </strong>Twenty subjects 9-19 years of age with pSLE underwent ABPM, peripheral endothelial function testing, carotid-femoral pulse wave velocity/analysis for aortic stiffness, and carotid intima-media thickness. We assessed the prevalence of non-dipping and other ABPM abnormalities. Pearson or Spearman rank correlation tests were used to evaluate relationships between nocturnal BP dipping, BP load (% of abnormally elevated BPs over 24-h), and vascular outcome measures.</p>

<p><strong>RESULTS: </strong>The majority (75%) of subjects had inactive disease, with mean disease duration of 3.2 years (± 2.1). The prevalence of non-dipping was 50%, which occurred even in the absence of nocturnal or daytime hypertension. Reduced diastolic BP dipping was associated with poorer endothelial function (r 0.5, p = 0.04). Intima-media thickness was significantly greater in subjects with non-dipping (mean standard deviation score of 3.0 vs 1.6, p = 0.02). In contrast, higher systolic and diastolic BP load were associated with increased aortic stiffness (ρ 0.6, p = 0.01 and ρ 0.7, p &lt; 0.01, respectively), but not with endothelial function or intima-media thickness.</p>

<p><strong>CONCLUSION: </strong>In a pSLE cohort with low disease activity, isolated nocturnal BP non-dipping is prevalent and associated with endothelial dysfunction and atherosclerotic changes. In addition to hypertension assessment, ABPM has a promising role in risk stratification and understanding heterogeneous mechanisms of cardiovascular disease in pSLE.</p>

<p><strong>OBJECTIVES: </strong>The increased relative risk of heart failure (HF) from systemic lupus erythematosus (SLE) is greatest at younger ages, but the etiology remains unclear. We identified risk factors for HF in children and adults with SLE and evaluated associations between SLE manifestations and HF.</p>

<p><strong>METHODS: </strong>Incident SLE cases without preceding HF were identified using Clinformatics DataMart® (OptumInsight, Eden Prairie, MN) US claims data (2000-2015), and categorized by age of SLE onset (children 5-17, young adults 18-24, adults 25-44 years old). The primary outcome was the first HF ICD-9-CM diagnosis code (428.x), categorized as early-onset (&lt; 6 months) or delayed-onset. Multivariable logistic regression was used to identify factors associated with early or delayed-onset HF. Cox proportional hazards regression was used to identify time-dependent associations between the onset of SLE manifestations and incident HF.</p>

<p><strong>RESULTS: </strong>There were 523 (2.3%) HF cases among 1,466 children, 2,163 young adults and 19,349 adults age 25-44 with SLE. HF in children and young adults was early-onset in 50% and 60% of cases, respectively, compared to 35% of cases in adults 25-44 years old. There was a temporal association between incident myopericarditis and valvular disease diagnoses and early-onset HF, whereas nephritis and hypertension were more strongly associated with delayed-onset HF. Black race remained independently associated with a 1.5-fold increased HF risk at any time.</p>

<p><strong>CONCLUSION: </strong>Hypertension remains an important traditional CV risk factor across all ages and should be managed aggressively even in younger patients with SLE. Cardiac dysfunction due to acute cardiac manifestations of SLE may contribute to the very high relative incidence of early HF diagnoses among younger SLE patients. Therefore, future prospective studies will need to address heterogeneity in the types and severity of heart failure in order to determine etiology and which patients should be monitored.</p>

<p><strong>OBJECTIVE: </strong>Youth with systemic lupus erythematosus (SLE) transferring from pediatric to adult care are at risk for poor outcomes. We describe patterns of rheumatology/nephrology care and changes in health care utilization and medication adherence during transfer.</p>

<p><strong>METHODS: </strong>We identified youth ages 15-25 with SLE using US private insurance claims from Optum's de-identified Clinformatics® Data Mart. Rheumatology/nephrology visit patterns were categorized as 1) unilateral transfers to adult care within 12 months, 2) overlapping pediatric and adult visits, 3) lost to follow-up, or 4) continuing pediatric care. We used negative binomial regression and paired t-tests to estimate changes in health care utilization and medication possession ratios (MPR) after the last pediatric (index) visit. We compared MPRs between youth who transferred and age-matched peers continuing pediatric care.</p>

<p><strong>RESULTS: </strong>184 youth transferred out of pediatric care, of which 41.8% transferred unilaterally, 31.5% had overlapping visits over a median of 12 months before final transfer, and 26.6% were lost to follow-up. We matched 107 youth continuing pediatric care. Overall ambulatory utilization decreased among those lost to follow-up. Acute care utilization decreased across all groups. MPRs after the index date were lower in youth lost to follow-up (mean 0.24) compared to peers in pediatric care (0.57, p&lt;0.001).</p>

<p><strong>CONCLUSION: </strong>Youth with SLE with continuous private insurance coverage do not use more acute care after transfer to adult care. However, a substantial proportion fail to see adult subspecialists within 12 months and have worse medication adherence, placing them at higher risk for adverse outcomes.</p>

<p><strong>OBJECTIVE: </strong>Mental health problems are prevalent in youth with rheumatologic disease. Gaps in knowledge exist regarding their impact, as well as strategies for detection and effective treatment. To address these gaps, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Mental Health Workgroup developed and prioritized an agenda of research topics.</p>

<p><strong>METHODS: </strong>We systematically reviewed the literature and identified 5 major research domains in further need of study: (A) mental health burden and relationship to pediatric rheumatologic disease, (B) impact of mental health disorders on outcomes, (C) mental health awareness and education, (D) mental health screening, and (E) mental health treatment. Research topics within these areas were developed by workgroup leaders and refined by the workgroup. Members were surveyed to prioritize the topics by importance, feasibility of study, and actionability.</p>

<p><strong>RESULTS: </strong>Fifty-nine members (57%) completed the survey. Among the proposed research topics, 31/33 were rated as highly important and 4/33 were rated highly for importance, feasibility, and actionability. Topics rated most important related to (A) mental health burden and relationship to rheumatologic disease, and (B) the impact of mental health on outcomes. Topics rated most feasible and actionable were related to (D) mental health screening.</p>

<p><strong>CONCLUSION: </strong>Addressing gaps in knowledge regarding mental health in youth with rheumatologic disease is essential for improving care. We have identified high priority research topics regarding mental health of pediatric rheumatology patients in need of further investigation that are feasible to study and believed to lead to actionable results in patient care.</p>

<p>Depression and anxiety are prevalent in children with rheumatologic diseases, including juvenile idiopathic arthritis (JIA). However, prevalence rates and the relationship with disease outcomes, including quality of life are conflicting in the early literature. To review the current literature, determine gaps in our knowledge, and identify areas in need of further investigation, we conducted a systematic review of studies examining depression and anxiety symptoms among children with JIA and the impact these symptoms may have on disease outcomes and quality of life. Six electronic databases were searched up until January 2019. Of 799 potential articles, 60 articles were included with the main focus on 28 articles from 2009 to 2019, to concentrate on the most current evidence. We found that JIA patients experience symptoms of depression and anxiety similar to other childhood chronic diseases and at higher rates than in healthy children. Patients who experience these symptoms have worse quality of life, with some evidence pointing to depression and anxiety symptoms having a greater impact on quality of life than other disease features, such as active joint count. Family members of JIA patients experience high rates of anxiety and depression symptoms which may impact their child's mental health and pain symptoms related to JIA. Conflicting reports of associations between depression/anxiety symptoms and disease features/disease outcomes and a paucity of longitudinal studies investigating the impact of treatment on mental health symptoms indicate areas in need of further research to effectively identify patients at greatest risk of depression and anxiety and to better understand how to treat and prevent these symptoms in youth with JIA. Family mental health should also be considered in investigations concerning mental health and disease outcomes of children with JIA.</p>

<p>The timing and etiology of diastolic impairment in pediatric-onset systemic lupus erythematosus (SLE) are poorly understood. We compared echocardiographic metrics of left ventricular diastolic function in children at SLE diagnosis to controls and identified factors associated with diastolic indices. Echocardiograms of children aged 5-18&nbsp;years within 1&nbsp;year of SLE diagnosis and age-/sex-matched controls were retrospectively read by blinded cardiologists. Clinical characteristics were abstracted separately. Z-scores for diastolic indices (E/A, e', E/e', and isovolumetric relaxation time (IVRT)) were calculated using published normative data and study controls, and compared using linear mixed-effects models adjusted for blood pressure. Pericardial effusions and valvular disease were also evaluated. Linear regression was used to identify factors associated with diastolic measures. 85 children with incident SLE had echocardiograms performed a median of 6&nbsp;days after diagnosis (interquartile range (IQR) 1-70). Prior cumulative prednisone exposure was minimal (median 60&nbsp;mg, IQR 0-1652). SLE cases had lower E/A, lower e', higher E/e', and longer IVRT compared to controls. Though none met criteria for Grade I diastolic dysfunction, Z-scores for e', E/e', and IVRT were abnormal in 30%, 25%, and 6% of SLE cases, respectively. Greater disease activity was associated with lower septal e' (p &lt; 0.01), higher E/e' (p = 0.02), and longer IVRT (p &lt; 0.01). Children with incident SLE have worse diastolic indices at diagnosis compared to peers without SLE, independent of blood pressure and prior to significant prednisone exposure. Longitudinal studies will determine whether diastolic dysfunction develops in this population over time.</p>

<p>Parry-Romberg syndrome (PRS) is characterized by progressive atrophy of facial skin, soft tissues, muscle, and bone. En coup de sabre syndrome is a form of linear scleroderma (LS) involving the skin of the frontoparietal forehead and scalp. Both conditions can be associated with neurologic findings, including seizures. We explore a case in which skin findings and seizure burden improved with methotrexate therapy.</p>

<p><strong>Objective: </strong>Thirty percent of adults with fibromyalgia receive an opioid, but the prevalence of opioid prescribing in pediatric chronic musculoskeletal pain is unknown. The aims of this study were to determine the prevalence of and factors associated with opioid exposure and polypharmacy among children with chronic musculoskeletal pain.</p>

<p><strong>Methods: </strong>In this retrospective cohort study using health care claims data from 2000 to 2013, the index date was the first ICD-9 code 729.1. Included subjects were ≥ 2 and &lt; 18 years old at the index date with two or more codes within 12 months and 18 months of continuous enrollment. Subjects with burns, sickle cell disease, or malignancy were excluded. Opioid exposure was defined as one or more prescriptions within six months before or any time after the index date. Polypharmacy was considered minor (2-4 medications) or major (≥5 medications).</p>

<p><strong>Results: </strong>Of 25,321 included subjects, 20% received an opioid and 26% experienced minor polypharmacy. Opioid exposure was associated with female sex (odds ratio [OR] = 1.27, P &lt; 0.01), Caucasian race (OR = 1.27, P &lt; 0.01), hospitalization (OR = 1.20, P &lt; 0.01), and visit with anesthesiology (OR = 1.97, P &lt; 0.01) or orthopedics (OR = 1.09, P &lt; 0.05). Mental health codes were associated with decreased odds of opioid exposure (all P &lt; 0.05). Children seen by a chiropractor or physiatrist had a reduced odds of receipt of an opioid (OR = 0.42 and 0.84, respectively, both P &lt; 0.01).</p>

<p><strong>Conclusions: </strong>Twenty percent of children with chronic musculoskeletal pain received an opioid. Twenty-six percent experienced polypharmacy, with the majority receiving 2-4 medications. Increased availability of psychological and nonpharmacologic services are potential strategies to reduce opioid exposure.</p>