Deborah W. Kredich

Chimerism in children with juvenile dermatomyositis

Juvenile dermatomyositis is a disease with similarities to chronic graft-versus-host disease. To identify whether chimerism is present in juvenile dermatomyositis, we investigated the families of 15 children with the disorder. Chimerism was identified by PCR in 13 of the 15 affected children, compared with five of 35 siblings (p<0.0001). Maternal cells among peripheral-blood mononuclear cells were detected in 11 of the 15 boys, compared with five of 17 unaffected controls (p=0.02), and in muscle tissue of 12 of 15 compared with two of ten unaffected siblings (p=0.005). These results suggest that chimerism may be involved in juvenile dermatomyositis.

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.

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.

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.

Expression of IgM and IgG autoantibodies in pediatric and adult systemic lupus erythematosus

To compare patterns of autoantibody responses in pediatric and adult patients with systemic lupus erythematosus (SLE). IgG and IgM antibodies to single-stranded DNA (ssDNA), Sm, and the 70-kDa protein component of the RNP antigen (70-kDa RNP) were measured in 29 pediatric and 36 adult patients by enzyme-linked immunosorbent assays. Antibodies of either isotype to ssDNA, Sm, and 70-kDa RNP were present in 64, 58, and 79% of pediatric patients, respectively, comparable to prevalences of these autoantibodies in the adult SLE patients. Pediatric SLE patients were more likely than adult patients to have IgM anti-Sm antibodies (41.4% vs 13.9%, P = 0.02) and tended to more commonly express IgM anti-70-kDa RNP and IgM anti-ssDNA antibodies. The prominence of IgM autoantibody responses among pediatric SLE patients was shown by multiple logistic regression analysis to be related to total IgM concentrations and not related to age or duration of disease. Sequential serum samples available from several pediatric patients revealed the maintenance of similar patterns of isotype responses over time in approximately one-half of patients. In those patients whose responses changed over time, the variations in isotype expression were consistent with maturation of antibody responses of each specificity. While these results demonstrate similarities in autoimmune reactivities between pediatric and adult SLE patients, the serologic study of pediatric patients may provide an opportunity to more readily investigate the evolution of autoantibody responses.

Chronic Arthritis in Childhood

Chronic arthritis in childhood takes a number of different forms, each with its own distinctive potential complications and implications for prognosis. This article describes each of these forms, as well as therapeutic choices.