Family pain history predicts child health status in children with chronic rheumatic disease.

OBJECTIVES: To examine the relationships of parental and family pain history on the pain experience of children with chronic rheumatic disease. The aims of the study were as follows: 1) to describe the pain history of parents and families of children with rheumatic disease, 2) to examine relationships between parental and family pain history and the pain report and physician-rated health status of children with chronic rheumatic disease, and 3) to determine whether child coping mediates the relationship between family pain history and the child's pain and physician-rated health status. METHOD: Parents of 100 children were recruited from a pediatric rheumatology clinic during routine visits. Parents completed questionnaires assessing parental pain history and family characteristics. Children in the study completed a series of questionnaires to assess pain and pain coping strategies, including the Coping Strategies Questionnaire and parts of the Pediatric Pain Questionnaire. A pediatric rheumatologist provided a global assessment of disease severity on a 100-mm visual analog scale as an index of child health status. RESULTS: A high number of parents of children seen in a pediatric rheumatology clinic described a personal pain history. More than 90% of parents reported having at least 1 chronic pain condition, with an equal proportion reporting an episode of pain in the past month. The most commonly reported pain conditions were lower back pain, shoulder/neck pain, and migraine headache pain. On average, this group of parents reported a history of 3.5 chronic pain conditions (standard deviation: 2.3) and reported having sought treatment for 1.7 (standard deviation: 2.3) of these conditions. Additionally, 93% of all parents reported extended family members experiencing at least 1 chronic pain condition. Correlational analyses indicated that parents reporting higher levels of current pain and higher mean levels of pain during the past month were more likely to have children reporting higher levels of current pain (r = 0.23 and r = 0.27). In addition, parents who sought more treatment for their own pain were more likely to have children reporting higher levels of pain (r = 0.22) and presenting with poorer health status (r = 0.22). Similarly, parents reporting higher levels of pain-related interference with activity were more likely to have children reporting higher levels of current pain (r = 0.23). Correlational analyses also indicated that children whose extended families reported a history of multiple pain conditions were more likely to report higher levels of current pain (r = 0.24) and more pain locations (r = 0.23). Finally, a series of mediational statistical models confirmed that child use of the pain coping strategy, catastrophizing, partially accounted for the relationship between several parent and family pain history variables and the child's own current pain ratings and physician global assessment. Specifically, child catastrophizing mediated the relationships between the total number of treated pain conditions and children's current pain ratings and physician global assessment. In addition, child catastrophizing was shown to mediate the relationship between parental mean level of pain in the past month and children's current pain rating and the relationship between total number of family pain conditions and children's current pain rating. Taken together, our results suggest that parental and familial pain experiences predict children's use of catastrophizing to cope with pain, which in turn predicts physician global assessment and children's current pain. CONCLUSIONS: The results from the present study indicate that many of the parents of children seen in a pediatric rheumatology clinic have a personal pain history and highlight the potential impact of parental pain history on children's pain experiences. Specifically, parents who were more likely to seek treatment for their own pain or more likely to report interference with recreational activities because of pain had children with higher pain ratings and poorer health status as measured by the physician global assessment. Additionally, a series of mediational models showed that child catastrophizing serves as a specific mechanism through which parental and familial pain history variables influence child ratings of current pain and physician ratings of health status. Future studies are needed to determine exactly how children living in families with painful conditions become more reliant on catastrophizing to cope with their pain. In addition, more research is needed to identify other potential mediators, such as positive ways parents may influence children's pain coping. There are several important clinical implications of our findings. First, our results suggest that by gathering information from parents about their own pain histories, health care providers may be able to identify children at risk for developing maladaptive pain coping strategies and higher levels of disease-related pain and disability. Second, our results indicate that intervention programs should focus specifically on reducing children's use of catastrophizing to cope with their pain. Perhaps most importantly, our results highlight the need to include parents in interventions aimed at reducing children's pain and improving children's abilities to cope with pain.

Effects of inherited thrombophilic mutations in an adolescent with antiphospholipid syndrome and systemic lupus erythematosus.

Thrombophilia can result from either inherited or acquired conditions. We describe a teenager who developed extensive thrombosis requiring aggressive and prolonged anticoagulation. Laboratory evaluation revealed an acquired lupus anticoagulant, consistent with the antiphospholipid antibody syndrome (APS). DNA analysis revealed inherited thrombophilic mutations in the factor V and methylene tetrahydrofolate reductase genes. We believe that the combination of inherited and acquired hypercoagulable conditions affected her therapeutic response to anticoagulant therapy. Inherited thrombophilic DNA mutations may contribute to the hypercoagulability observed in patients with acquired thrombophilic conditions such as APS and systemic lupus erythematosus.

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 Student's 2-sided t-test) and prescriptions for shoe lifts (P = 0.002, by Fisher's 2-sided exact test). There was not a significant difference in TCD between the 2 groups (P = 0.139, by Student's 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.

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.

The first certifying examination in pediatric rheumatology.

OBJECTIVE: On May 12, 1992, the first certifying examination in pediatric rheumatology was administered by the American Board of Pediatrics to 94 candidates. We describe the certifying process and the characteristics of the examinees. METHODS: The Sub-board of Pediatric Rheumatology developed an examination consisting of 235 multiple choice items covering a broad range of topics pertinent to the subspecialty. Applicants with a current certificate in general pediatrics and a current medical license were admitted through one of 3 pathways requiring 2 years of fellowship training or equivalent practice experience. RESULTS: Sixty-three percent of the candidates had completed at least 2 years of fellowship training. The total group average score was 76% correct. Eighty-five percent of the examinees passed the examination. CONCLUSION: The 80 candidates who were certified received time limited certificates that may be renewed by a recertification process to begin in 1999. A second certifying examination was offered in 1994 and a third in 1996. Requirements for future certifying examinations will include 3 years of full time fellowship training and verification of meaningful accomplishment in research.

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. Pearson's 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.

Breast feeding and the development of juvenile rheumatoid arthritis.

OBJECTIVE: To determine if children with juvenile rheumatoid arthritis (JRA) are less likely to have been breast fed than controls. METHODS: Case-control study of data obtained from a survey of mothers 54 children with JRA and 79 playmates regarding breast feeding. Duration of breast feeding was tabulated and odds ratios (OR) with 95% confidence intervals (CI) were determined. RESULTS: OR for breast feeding in children with JRA was 0.40 (0.20-0.81, 95% CI) compared to playmates. For pauciarticular JRA (N = 28) OR was 0.31 (0.10-0.93); in polyarticular JRA (N = 24) OR was 0.60 (0.21-1.70). Lower OR for increased durations of breast feeding were noted in children with JRA. CONCLUSION: Children who have had JRA, especially pauciarticular JRA, are less likely to have been breast fed than controls, suggesting that breast feeding may have a protective effect on the development of JRA.

Disease experience and psychosocial adjustment in children with juvenile rheumatoid arthritis: children's versus mothers' reports.

Adjustment in children with juvenile rheumatoid arthritis (JRA) has been evaluated most frequently by parental or teacher reports and with reference to disease severity. In this study, 38 children, ages 7 to 13 with JRA, and their mothers were interviewed. Modest correlations were found between children and mothers in their assessments of children's perceived competence in several domains (i.e., athletic competence, social acceptance, physical attractiveness, and global self-worth) and in their perceptions of how JRA is experienced by children and families. Children's perceptions of the disease experience were significantly correlated with the four measures of perceived competence, even after controlling for disease severity. The results highlight the importance of cross-validating parental reports with children's self-reports, and demonstrate the need to consider variables other than disease severity--in particular how JRA is interpreted by children--in predicting their adjustment.

Auranofin therapy for juvenile rheumatoid arthritis: results of the five-year open label extension trial.

Eighty-eight children with juvenile rheumatoid arthritis (JRA) who completed a double blind, randomized placebo controlled trial of oral gold were entered into an open label extension phase during which they received auranofin (AF) at a dosage of 0.15-0.2 mg/kg/day (9 mg/day maximum). Eleven (12.5%) patients completed 5 years of AF therapy; 77 (87.5%) did not. Fifteen (17%) of the 88 were in disease remission at the final visit. Mean duration of therapy for those who discontinued was 646 days. Parental/patient decision and insufficient therapeutic effect were the 2 most frequent reasons for early termination, followed by adverse effects. Though relatively well tolerated, AF provides adequate longterm management for only a small percentage of patients with JRA.

Pediatric spondyloarthropathies.

Seronegative spondyloarthropathies in childhood are often misdiagnosed as juvenile rheumatoid arthritis, but recognition of their distinct clinical manifestations and unique underlying pathophysiologies can aid in making a proper diagnosis. Ankylosing spondylitis, Reiter's syndrome, psoriatic arthritis, and the arthritis associated with inflammatory bowel disease are arthritides most often found in young adults, but they may also be present in children. Extraarticular manifestations include inflammation of the eyes, skin, gastrointestinal tract, and genitourinary tract associated with inflammation of the entheses. The proper diagnosis will allow for treatment regimens that differ from those usually used for juvenile rheumatoid arthritis. Early diagnosis and treatment often lead to an early recovery and a return to normal daily activities.

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.

Patterns of autoantibody expression in pediatric and adult systemic lupus erythematosus.

To characterize patterns of autoantibody expression in pediatric and adult systemic lupus erythematosus (SLE), IgG and IgM antibodies to cardiolipin (aCL) and single-stranded DNA (anti-DNA) were measured in sera from 32 pediatric and 36 adult patients. Antibody levels were assessed by isotype-specific ELISA and compared with 15 pediatric disease controls and 36 healthy adult controls. These determinations revealed significant differences in the pattern of IgG and IgM autoantibody expression in adult and pediatric patients. IgM aCL were more common in the pediatric than adult SLE population with 11 of 32 pediatric patients being positive compared to only 4 of 36 in the adult group (p less than 0.05). Conversely, enhanced IgG autoantibody production was observed in the adult group as 27 of 36 adult patients were positive for IgG anti-DNA compared to only 16 of 32 children (p less than 0.05). Analysis of sequential sera showed differences in the temporal expression of IgG and IgM autoantibodies as well as variability between anti-DNA and aCL responses over time. Our data suggest differences in the isotype profile of autoantibody responses in pediatric and adult SLE and provide further evidence for heterogeneity in the mechanisms of autoantibody production.

The relationship of anticardiolipin antibodies to disease manifestations in pediatric systemic lupus erythematosus.

To determine the prevalence of anticardiolipin antibodies (aCL) in pediatric systemic lupus erythematosus (SLE) and their possible association with clinical manifestations, aCL were measured in sera of 32 patients with the onset of SLE before age 16. IgM and IgG aCL were determined by ELISA and values compared to those of 12 patients with juvenile rheumatoid arthritis (JRA), 15 age matched asthmatics, and 32 adult controls. aCL were demonstrated in sera of 16 of 32 (50%) children with SLE, 5 of 12 (42%) patients with JRA, 1 of 15 (7%) asthmatics, and in none of the 32 adult controls. Serial samples on 11 patients with SLE showed fluctuations in aCL levels that often corresponded to disease activity; the highest levels occurred in patients during periods of seizure activity and other neurologic events. The antibodies were not crossreactive anti-DNA antibodies as shown by the failure of DNA to inhibit binding to cardiolipin. These data suggest that the prevalence of aCL is similar in pediatric and adult SLE and that aCL levels may vary with disease activity, especially neurologic disease.

Flurbiprofen in the treatment of juvenile rheumatoid arthritis.

Thirty-four patients with juvenile rheumatoid arthritis, who were treated with flurbiprofen at a maximum dose of 4 mg/kg/day, had statistically significant decreases from baseline in 6 arthritis indices after 12 weeks of treatment. Improvements were seen in the number of tender joints, the severity of swelling and tenderness, the time of walk 50 feet, the duration of morning stiffness and the circumference of the left knee. The most frequently observed side effect was fecal occult blood (25% of patients); however, there was no other evidence of gastrointestinal (GI) bleeding in these patients. One patient was prematurely discontinued from the study for severe headache and abdominal pain. Most side effects were mild and related to the GI tract.

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.