Laura E. Schanberg

Prevention of leg length discrepancy in young children with pauciarticular juvenile rheumatoid arthritis by treatment with intraarticular steroids

OBJECTIVE To determine if intraarticular (i.a.) injection of triamcinolone hexacetonide (steroids) used early in the course of pauciarticular juvenile rheumatoid arthritis (pauci JRA) is associated with less leg length discrepancy (LLD) or thigh circumference discrepancy (TCD). METHODS Children with pauci JRA who had asymmetric lower-extremity arthritis diagnosed before age 7 years in Seattle, Washington (WA; n = 16) and in Chapel Hill and Durham, North Carolina (NC; n = 14) were retrospectively identified. WA children were given i.a. steroids within 2 months of diagnosis; the injections were repeated if synovitis recurred in the same joint or in a different joint. These children were compared with NC children who were not treated with i.a. steroids. Thigh circumference was measured at 10 cm above the patella, and leg length was measured from the anterior superior iliac spine to the mid-medial malleolus, by a single observer. LLD and TCD are reported as the percentage of difference between leg measurements in each subject. RESULTS The WA and NC subjects had comparable disease severity and duration of followup (in months). Twelve WA children had subsequent i.a. steroid injections (mean 3.25 injections per child over mean +/- SD 42 +/- 11 months). The WA subjects had significantly less LLD (P = 0.005, by Students 2-sided t-test) and prescriptions for shoe lifts (P = 0.002, by Fishers 2-sided exact test). There was not a significant difference in TCD between the 2 groups (P = 0.139, by Students 2-sided t-test). Similar findings were obtained when the analysis was limited to children with monarticular knee arthritis. CONCLUSION Early and continued use of i.a. steroids may be associated with less LLD in young children with pauci JRA. This may indicate decreased duration of synovitis.

Pain coping and the pain experience in children with juvenile chronic arthritis.

&NA; This study examined the pain experience and pain coping of children with juvenile chronic arthritis (JCA). The purpose of the study was to describe present pain and the pain coping strategies utilized by children with juvenile chronic arthritis and examine pain coping strategies and pain efficacy as a predictor of pain intensity and distribution. Fifty‐six children with JCA rated their present pain using two measures of pain intensity, the Oucher and the pain thermometer, and reported on the number of pain locations using a body map. In addition, each child completed the Child Version of the Coping Strategies Questionnaire (CSQ‐C) and health status was determined by a physicians disease activity rating. On average, children reported current pain in the low to middle range on the different pain scales, although there was considerable variability in pain ratings. Up to 30% of all children had pain ratings higher than or equal to the middle range on both the Oucher and the pain thermometer. On average, children reported pain in more than two body areas. Correlational analyses were conducted to examine how the composite factors on the CSQ‐C (Pain Control and Rational Thinking, and Coping Attempts) related to variations in reported pain intensity and location. Children who scored higher on the Pain Control and Rational Thinking factor of the CSQ‐C had much lower ratings of pain intensity and reported pain in fewer body areas. Hierarchical regression analyses indicated that disease activity and scores on the Pain Control and Rational Thinking factor of the CSQ‐C each accounted for a unique, statistically significant proportion of variance in the measures of pain intensity and pain location. Behavioral and cognitive therapy interventions designed to increase pain coping efficacy may be useful adjuncts in treating pain in children with chronic arthritis.

Premature atherosclerosis in pediatric systemic lupus erythematosus: Risk factors for increased carotid intima‐media thickness in the atherosclerosis prevention in pediatric lupus erythematosus cohort

OBJECTIVE To evaluate risk factors for subclinical atherosclerosis in a population of patients with pediatric systemic lupus erythematosus (SLE). METHODS In a prospective multicenter study, a cohort of 221 patients underwent baseline measurements of carotid intima-media thickness (CIMT) as part of the Atherosclerosis Prevention in Pediatric Lupus Erythematosus (APPLE) trial. SLE disease measures, medications, and traditional risk factors for atherosclerosis were assessed. A standardized protocol was used to assess the thickness of the bilateral common carotid arteries and the mean maximal IMT of 12 segments. Univariable analysis identified potential associations with CIMT, which were examined in multivariable linear regression modeling. RESULTS Based on the mean-mean common or the mean-max CIMT as the dependent variable, univariable analysis showed significant associations of the following variables with increased CIMT: increasing age, longer SLE duration, minority status, higher body mass index (BMI), male sex, increased creatinine clearance, higher lipoprotein(a) level, proteinuria, azathioprine treatment, and prednisone dose. In multivariable modeling, both azathioprine use (P=0.005 for the mean-mean model and P=0.102 for the mean-max model) and male sex (P<0.001) were associated with increases in the mean-max CIMT. A moderate dosage of prednisone (0.15-0.4 mg/kg/day) was associated with decreases in the mean-max CIMT (P=0.024), while high-dose and low-dose prednisone were associated with increases in the mean-mean common CIMT (P=0.021) and the mean-max CIMT (P=0.064), respectively. BMI (P<0.001) and creatinine clearance (P=0.031) remained associated with increased mean-mean common CIMT, while increasing age (P<0.001) and increasing lipoprotein(a) level (P=0.005) were associated with increased mean-max CIMT. CONCLUSION Traditional as well as nontraditional risk factors were associated with increased CIMT in this cohort of patients in the APPLE trial. Azathioprine treatment was associated with increased CIMT. The relationship between CIMT and prednisone dose may not be linear.

The relationship of daily mood and stressful events to symptoms in juvenile rheumatic disease

OBJECTIVES The purpose of this study was 3-fold: 1) to assess the feasibility of a daily diary for use with children with juvenile rheumatic disease (JRD), 2) to describe daily variation in mood, stressful events, and symptoms in children with JRD, and 3) to examine the extent to which daily mood and daily stressful events predict daily symptoms in children with JRD. METHODS Twelve children with JRD completed a daily booklet for 7 days. The daily booklet included measures of daily mood, daily stressful events, daily symptoms, and daily function. The children also completed a visual analog scale for pain and the Childrens Depression Inventory. RESULTS Subjects showed good compliance with scheduled completion and return of the daily diaries. Results indicated that children with JRD showed variability in daily mood, frequency of daily stressful events, and daily symptoms across days. Multilevel fixed effects models showed that more negative daily mood and more daily stressful events significantly predicted increased reports of fatigue, stiffness, and cutting back on daily activities. Negative daily mood also correlated with increases in daily reported pain. CONCLUSIONS These results indicate that daily diary research is both feasible and potentially informative in children with JRD. Our data emphasize the need for further investigation into the role of daily mood and daily stressful events on disease course in JRD.

Consensus treatment plans for induction therapy of newly diagnosed proliferative lupus nephritis in juvenile systemic lupus erythematosus

To formulate consensus treatment plans (CTPs) for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in juvenile systemic lupus erythematosus (SLE).

Social context of pain in children with Juvenile Primary Fibromyalgia Syndrome : Parental pain history and family environment

OBJECTIVE The purpose of this study was to describe parental pain history and the family environment as it relates to the functional status of children with Juvenile Primary Fibromyalgia Syndrome (JPFS). DESIGN AND OUTCOME MEASURES Twenty-nine parents of children with JPFS completed a pain history questionnaire, Von Korff Chronic Pain Grading system, and the Family Environment Scale (FES). Twenty-one adolescents with JPFS completed the FES, the Visual Analogue Scale for Pain, the modified Fibromyalgia Impact Questionnaire for Children, the Arthritis Impact Measurement Scales, and the Symptom Checklist-90-Revised. Correlational analyses were performed. RESULTS Parents of children with JPFS reported multiple chronic pain conditions, including but not limited to fibromyalgia. Parental pain history and the family environment correlated with the health status of adolescents with JPFS. Children with JPFS perceived the family environment as significantly more cohesive than did their parents. Greater incongruence between parent and child responses on the FES positively correlated with greater impairment. CONCLUSIONS These results suggest that family environment and parental pain history ày be related to how children cope with JPFS. Behavioral interventions targeting the family may improve the long-term functional status of children with JPFS.

Use of Atorvastatin in Systemic Lupus Erythematosus in Children and Adolescents

OBJECTIVE Statins reduce atherosclerosis and cardiovascular morbidity in the general population, but their efficacy and safety in children and adolescents with systemic lupus erythematosus (SLE) are unknown. This study was undertaken to determine the 3-year efficacy and safety of atorvastatin in preventing subclinical atherosclerosis progression in pediatric-onset SLE. METHODS A total of 221 participants with pediatric SLE (ages 10-21 years) from 21 North American sites were enrolled in the Atherosclerosis Prevention in Pediatric Lupus Erythematosus study, a randomized double-blind, placebo-controlled clinical trial, between August 2003 and November 2006 with 36-month followup. Participants were randomized to receive atorvastatin (n=113) or placebo (n=108) at 10 or 20 mg/day depending on weight, in addition to usual care. The primary end point was progression of mean-mean common carotid intima-media thickening (CIMT) measured by ultrasound. Secondary end points included other segment/wall-specific CIMT measures, lipid profile, high-sensitivity C-reactive protein (hsCRP) level, and SLE disease activity and damage outcomes. RESULTS Progression of mean-mean common CIMT did not differ significantly between treatment groups (0.0010 mm/year for atorvastatin versus 0.0024 mm/year for placebo; P=0.24). The atorvastatin group achieved lower hsCRP (P=0.04), total cholesterol (P<0.001), and low-density lipoprotein (P<0.001) levels compared with placebo. In the placebo group, CIMT progressed significantly across all CIMT outcomes (0.0023-0.0144 mm/year; P<0.05). Serious adverse events and critical safety measures did not differ between groups. CONCLUSION Our results indicate that routine statin use over 3 years has no significant effect on subclinical atherosclerosis progression in young SLE patients; however, further analyses may suggest subgroups that would benefit from targeted statin therapy. Atorvastatin was well tolerated without safety concerns.

Pain coping strategies in children with juvenile primary fibromyalgia syndrome: Correlation with pain, physical function, and psychological distress

OBJECTIVES The purpose of this study was twofold: 1) to describe the coping strategies used by children with juvenile primary fibromyalgia syndrome (JPFS), and 2) to examine how pain coping relates to measures of pain, disability/function, psychological distress, and pain behavior. METHODS Sixteen children with JPFS completed the Child Version of the Coping Strategies Questionnaire (CSQ-C), the visual analog scale for pain, the McGill Pain Questionnaire, the Fibromyalgia Impact Questionnaire modified for children, the Arthritis Impact Measurement Scales 2, and the Symptom Checklist-90-Revised. Subjects also also underwent pain behavior observation. Pearsons product moment correlations were conducted to examine the relationship of coping to measures of pain and disability. RESULTS The Pain Control and Rational Thinking composite factor score on the CSQ-C correlated with measures of pain severity, functional disability, and psychological distress. Results supported the internal reliability of the CSQ-C in assessing pain coping. CONCLUSIONS These results suggest that the CSQ-C may provide a reliable measure for assessing variations in pain coping in JPFS patients. Behavioral interventions aimed at increasing the perception of pain control may be beneficial in treating JPFS.

A systematic review of sleep in pediatric pain populations

Objective: The primary aim of this systematic review was to examine the evidence for a pain-sleep relationship in children with persistent pain by reviewing studies using single and mixed pediatric persistent pain samples. Methods: Electronic searches of Medline, PubMed, the Cochrane Database of Systematic Reviews, and PsycINFO were conducted to identify all relevant empirical studies. Studies were included in the review if the majority of participants were between 0 and 17 years and from one of the following pediatric pain populations: juvenile idiopathic arthritis, sickle cell disease, migraine/headache, functional abdominal pain, juvenile fibromyalgia syndrome, chronic musculoskeletal pain, or mixed populations including the aforementioned conditions. Results: Research from single and mixed sample studies support the hypothesis that children and adolescents with persistent pain suffer from sleep impairment. Literature addressing factors that may influence or mediate the pain-sleep relationship and the functional outcomes of the pain-sleep relationship was reviewed, and a model of the interrelationships with pain and sleep was developed. Conclusion: Findings from this review highlight the need to assess and treat sleep problems in children presenting with persistent pain. Health care providers should consider conducting routine sleep screenings, including a comprehensive description of sleep patterns and behaviors obtained through clinical interview, sleep diaries, and/or the use of standardized measures of sleep. Future research focusing on investigating the mechanisms associating sleep and pediatric persistent pain and on functional outcomes of poor sleep in pediatric pain populations is needed.

Randomized, Double-Blind, Placebo-Controlled Trial of the Efficacy and Safety of Rilonacept in the Treatment of Systemic Juvenile Idiopathic Arthritis

To assess the efficacy and safety of rilonacept, an interleukin‐1 inhibitor, in a randomized, double‐blind, placebo‐controlled trial.