Agreement between parents and physicians regarding clinical evaluation of patients with juvenile rheumatoid arthritis

The reliability of disease severity assessed by parents of children with juvenile rheumatoid arthritis was studied by a self-report questionnaire. Parents of 50 patients with a minimum disease duration of 6 months and two physicians participated. Good agreement was achieved between parents and physicians regarding global assessment of disease severity, pain, morning stiffness and activities of daily life. For joint swelling and restriction of movement, the physicians scored higher than did the parents. The findings demonstrate that, with some reservations, a parent questionnaire may be used in follow-up studies on children with juvenile rheumatoid arthritis.     

Pain experience and pain coping strategies in children with juvenile idiopathic arthritis

OBJECTIVE: To compare reactions to cold pressor pain and pain coping strategies of patients with juvenile idiopathic arthritis (JIA), healthy children, and their parents. METHODS: We studied 16 children with JIA and one of their parents and 14 healthy children and one of their parents. Patients with JIA were selected from the patient population by fulfilling criteria for inclusion in a "high pain" group (n = 7) of patients with modest clinical arthritis activity, but who presented daily reports of pain in connection with everyday activities, and a "low pain" group (n = 9) who presented significant clinical arthritis activity, but who had only a few complaints of pain related to everyday activities. Dependent variables included pain threshold, discomfort, intensity and tolerance to cold pressor pain, and pain coping strategies. RESULTS: Patients with JIA exhibited significantly lower mean pain tolerance than healthy children. Disease duration correlated with both experimental and clinical pain measures, and JIA patients used significantly more Behavioral Distraction than healthy children. Correlations were found between children's and parents' use of Approach and Distraction related coping strategies. Correlations were also found for the coping strategy of Catastrophizing in the JIA patient group. For experimental pain coping strategies, a significant correlation was found between the JIA patients' and their parents' use of Distraction. For the JIA patients Positive Self-statements and Behavioral Distraction were inversely correlated with the clinical pain measures. In both children and parents the experimental pain coping strategies of Catastrophizing and Distraction were associated with the experimental pain response measures, and low pain JIA patients tended to use more Distraction pain coping strategies than high pain patients. CONCLUSION: The results indicate that JIA patients may differ from healthy children with regard to their responses to experimental pain as well as to their use of pain coping strategies. Pain coping strategies of JIA patients were associated with pain coping strategies of their parents, and use of pain coping strategies was associated with both experimental and clinical pain experience.     

Quality of life outcomes in congenital chronic intestinal pseudo-obstruction

The goal of this study was to assess the quality of life for children with chronic intestinal pseudoobstruction. We used a retrospective chart review to identify children with congenital chronic intestinal pseudoobstruction, then a structured telephone interview with parents that included the Child Health Questionnaire to gather information about the current status and quality of life for each patient and family. Children with chronic intestinal pseudo-obstruction had less freedom from pain, depression, and anxiety than healthy children or children with juvenile rheumatoid arthritis (P < 0.05 for all three parameters). Parents of children with chronic intestinal pseudo-obstruction had poorer emotional status than parents of healthy children or children with juvenile rheumatoid arthritis. The time required for parents to care for children with chronic intestinal pseudo-obstruction was greater than the time required to care for healthy children or children with juvenile rheumatoid arthritis (P < 0.01). In conclusion, the quality of life for children with chronic intestinal pseudo-obstruction lags behind that of healthy children and children with another chronic illness. Appropriate treatment of chronic pain may improve the quality of life for children with chronic intestinal pseudo-obstruction and their families. Moreover, attention to reducing each family's burden of time and emotional distress may help them cope better with their chronically ill child.     

Assessment of pain in patients with juvenile rheumatoid arthritis: relation between pain intensity and degree of joint inflammation.

The relation between pain and joint inflammation in patients with juvenile rheumatoid arthritis has not previously been systematically evaluated. Eighteen patients with juvenile rheumatoid arthritis completed paediatric pain questionnaires and the joints affected were examined by thermography. Although significant correlations were shown between parent and doctor pain intensity ratings and joint temperature, correlations of patient pain intensity ratings and joint temperature were only significant in younger children. The degree of joint inflammation is only one factor of several contributing to the amount of subjective pain experienced by children with juvenile rheumatoid arthritis, indicating the need for a comprehensive assessment of the relatively independent variables of inflammation and pain in children with juvenile rheumatoid arthritis.     

The relationship between health-related quality of life, pain and coping strategies in juvenile idiopathic arthritis

OBJECTIVES: To investigate the relationship between health-related quality of life (HRQL), experience of pain and pain coping strategies in children with juvenile idiopathic arthritis (JIA). To compare reports describing these variables obtained from children and their parents. METHODS: Participants were 59 children aged 8 to 18 yr with JIA and their parents. Parents and children completed the PedsQL generic core scales and arthritis module, the visual analogue scale of the Varni-Thompson Pediatric Pain Questionnaire, and the Waldron/Varni Pediatric Pain Coping Inventory. Parents rated children's functional disability using the Childhood Health Assessment Questionnaire. RESULTS: Parents reported significantly lower scores (indicating worse HRQL) than children on five of the eight PedsQL scales rating children's HRQL. Parents and children reported a significant negative relationship between pain levels and the PedsQL scores assessing children's physical, emotional and social functioning. They also reported a significant negative relationship between scores on several pain coping scales and scores on the PedsQL scales. However, the pattern of these relationships varied for reports from parents and children. CONCLUSIONS: Pain intensity and pain coping strategies have a significant and independent relationship with several domains that comprise the HRQL of children with JIA. However, parents and children have differing perceptions of the nature of these relationships. The differences emphasize the importance of clinicians obtaining information about children's HRQL, pain levels and pain coping strategies from both parents and children.     

Primary juvenile fibromyalgia. Psychological adjustment,family functioning,coping, and functional disability

Objectives. 1) To determine the importance of psychological adjustment and family functioning in primary juvenile fibromyalgia by assessing these factors in children with fibromyalgia and in their parents, compared with children with juvenile rheumatoid arthritis (JRA) and with pain-free control children and their parents. 2) To examine which of these factors predict functional disability. Methods. Fifteen children in each of the 3 study groups, and their parents, completed self-report questionnaires and pain diaries. A medical evaluation of each child was performed, including assessment of tender points by palpation and by dolorimetry. Results. All children in the fibromyalgia group met the Yunus and Masi criteria for fibromyalgia, and 11 met the American College of Rheumatology criteria. There were almost no significant group differences in the children's or parents' psychological adjustment, ratings of family functioning, or coping strategies. Significant group differences in functional disability, pain, fatigue, tender point threshold, and control point tolerance were found. A number of the psychological adjustment, pain, fatigue, and coping variables were significantly associated with functional disability. Conclusion. The notion that fibromyalgia is a psychogenic condition is not supported by these results. Fibromyalgia is associated with disability of a magnitude comparable to that of other chronic pain conditions. Disability among children with fibromyalgia or JRA is a function of the children's psychological adjustment and physical state, and of the parents' physical state and method of coping with pain.     

Frequencies of epstein-barr virus—inducible IgM anti-IgG B lymphocytes in normal children and children with juvenile rheumatoid arthritis

The relative frequencies of IgM antilgG autoantibody (rheumatoid factor) producing cells induced by the polyclonal B cell activator Epstein-Barr virus were measured in peripheral blood lymphocyte cultures of normal children and patients with juvenile rheumatoid arthritis. The frequencies of rheumatoid factor precursor B cells in normal children were lower than adults, but higher than neonates. The frequency increased with the age of the donor. In seronegative children with the systemic-onset or pauclarticular-onset types of juvenile rheumatoid arthritis, the number of IgM antiIgG inducible B cells was not significantly different (P>0.05) from age-matched controls. Patients with seropositive juvenile rheumatoid arthritis or seropositive adult rheumatoid arthritis had significantly higher IgM antiIgG precursor cell frequencies than age-matched normal subjects (P<0.01 and P<0.02, respectively). In contrast, the patients with seronegative polyarticular-onset juvenile rheumatoid arthritis had an average precursor frequency significantly lower than normal age-matched controls (P<0.05), analogous to results previously noted in adult seronegative rheumatoid arthritis. Thus, both children and adults with seronegative polyarticular rheumatoid arthritis had a deficiency in B cells that produce IgM antiIgG and that are induced by Epstein-Barr virus. This distinguished them from seropositive juvenile rheumatoid arthritis and rheumatoid arthritis patients, normal subjects, and patients with the pauciarticular-onset and systemic-onset types of seronegative juvenile rheumatoid arthritis.     

Diet and disease symptoms in rheumatic diseases — Results of a questionnaire based survey

Summary Experiences with food intake, diet manipulations and fast were registered in rheumatic patients. The study was a questionnaire-based survey in which 742 patients participated. It comprised 290 patients with rheumatoid arthritis, 51 patients with juvenile rheumatoid arthritis, 87 patients with ankylosing spondylitis, 51 patients with psoriatic arthropathy, 65 patients with primary fibromyalgia and 34 patients with osteoarthritis. One third of the patients with rheumatoid arthritis, ankylosing spondylitis and psoriatic arthropathy reported aggravation of disease symptoms after intake of certain foods while 43% of the patients with juvenile rheumatoid arthritis and 42% of the patients with primary fibromyalgia stated the same. Twenty-six percent of the patients with juvenile rheumatoid arthritis and 23% of the patients with rheumatoid arthritis, ankylosing spondylitis and primary fibromyalgia had previously tried certain diets in the attempt to alleviate disease symptoms, whereas 13% of the patients with sporiatic arthropathy and 10% with osteoarthritis had tried diet therapy. Less pain and stiffness were reported by 46% of the patients and 36% reported reduced joint swelling. Similar beneficial effects of diet were also reported in other rheumatic disease groups. Fifteen percent of the patients with rheumatoid arthritis and ankylosing spondylitis had been through a fasting period. Less pain and stiffness were reported by 2/3 of the patients in both groups and half of the patients in both groups reported a reduced number of swollen joints.     

Arthritis and the family

Objectives. This paper reviews the literature on arthritis and the family in two areas, juvenile rheumatic diseases and adult-onset arthritis. Methods. All published papers related to arthritis and the family were identified through a Medline search and through hand searching of the major rheumatology journals. Results. The literature on the family and juvenile arthritis is somewhat inconsistent, in that some studies demonstrate significant psychosocial impact among children and families, while others find no differences in children who have arthritis compared to normative data or to siblings. Lack of consistency in the literature is largely related to methodologic problems, as most studies are retrospective reports, consisting of relatively small, nonrepresentative samples of children with rheumatic diseases. The influence of adult-onset rheumatoid arthritis and osteoarthritis on family role functioning and the performance of household responsibilities is well documented, and a body of literature on the importance of family functioning to well-being is accumulating. Conclusions. Future studies should expand sample sizes in order to investigate family impact on juvenile rheumatoid arthritis more thoroughly. More studies on the factors that contribute to improved family functioning and subsequent improvements in well-being, especially in conditions other than rheumatoid arthritis and osteoarthritis, are needed. Intervention studies to reduce perceived pain and functional disability in arthritis by incorporating a family-systems perspective are particularly lacking.     

Update on the pathogenesis and treatment of systemic onset juvenile rheumatoid arthritis

PURPOSE OF REVIEW: Although systemic onset juvenile rheumatoid arthritis accounts for only about 20% of most reported series, children with systemic onset juvenile rheumatoid arthritis are often the most difficult to treat. Many children with persistent systemic onset juvenile rheumatoid arthritis have marked physical and emotional disability as a result of both disease and treatment-related morbidities. This review highlights recent studies that better elucidate the etiopathogenesis of systemic onset juvenile rheumatoid arthritis. New therapies derived from better understanding of cytokines, cytokine gene expression, and their complex interactions, which result in inflammation, are improving our ability to control active disease while reducing or reliance on corticosteroids. RECENT FINDINGS: Recent advances in our understanding of the etiopathogenesis of systemic onset juvenile rheumatoid arthritis have led to therapies that specifically target the cytokines found in abnormal quantities in children with active disease. Biologic agents that directly target interleukin-1a, interleukin-6, and tumor necrosis factor alpha are currently in use, and additional agents that modulate interleukin-18, myeloid-related proteins 8 and 14, natural killer cell function, and macrophage migration inhibitory factor production are under investigation. SUMMARY: Anakinra, monoclonal antibody to interleukin-6 receptor, and thalidomide each have led to significant clinical improvement with fewer side effects than resulted when corticosteroids were the mainstay of therapy.     

Worldwide prevalence of juvenile arthritis why does it vary so much?

OBJECTIVE: To review epidemiological studies of childhood arthritis from 1966, and to identify possible reasons for the wide-ranging results for both prevalence and incidence of juvenile arthritis (JA). JA is the term used here collectively for juvenile rheumatoid arthritis, juvenile chronic arthritis, or juvenile idiopathic arthritis as defined in the respective published studies. METHODS: A review of 34 epidemiological studies of JA since 1966 was undertaken. RESULTS: Prevalence of JA is reported as 0.07 to 4.01 per 1000 children. Annual incidence is reported as 0.008 to 0.226 per 1000 children. The major factors contributing to differences in estimates include (1) factors due to diagnostic difficulties, to the development of new diagnostic criteria, and to the differing definitions of clinical cases; (2) differences in case ascertainment (community based versus clinical case studies, qualification and experience of study clinicians, definition of study population); (3) factors occurring with the passage of time, i.e., standard of living, health care resources, and increasing knowledge; and (4) small studies and hence more chance fluctuation. The major variation in reported prevalence was due to the difference between true community based studies involving children from within classrooms or homes (and not necessarily previously diagnosed with JA) compared with clinical case studies of children who (by definition) had been previously diagnosed. The highest prevalence was reported for true community based studies. CONCLUSION: Many factors contribute to the discrepancies between reported prevalence and incidence for JA. Studies based truly in the community reported the highest prevalence, as previously undiagnosed cases were included. Future studies involving standardized criteria and standardized case ascertainment done by fully trained clinicians should show greater consistency of results.     

Development of diabetes mellitus during etanercept therapy in a child with systemic‐onset juvenile rheumatoid arthritis

Although clinical trials of etanercept in adult and juvenile rheumatoid arthritis have generally revealed few adverse events, significant concern has arisen over the potential evolution of secondary autoimmune disease due to modulation of tumor necrosis factor. There have been few reports of such diseases developing, and none in children receiving this therapy. Reported herein is the case of a 7‐year‐old girl with a 3‐year history of systemic‐onset juvenile rheumatoid arthritis with a polyarticular course, in whom type 1 diabetes mellitus developed 5 months after the initiation of etanercept therapy.     

Development of diabetes mellitus during etanercept therapy in a child with systemic-onset juvenile rheumatoid arthritis

Although clinical trials of etanercept in adult and juvenile rheumatoid arthritis have generally revealed few adverse events, significant concern has arisen over the potential evolution of secondary autoimmune disease due to modulation of tumor necrosis factor. There have been few reports of such diseases developing, and none in children receiving this therapy. Reported herein is the case of a 7-year-old girl with a 3-year history of systemic-onset juvenile rheumatoid arthritis with a polyarticular course, in whom type 1 diabetes mellitus developed 5 months after the initiation of etanercept therapy.     

Outcome following onset of juvenile idiopathic inflammatory arthritis: I. frequency of different outcomes

OBJECTIVE: To determine the outcome, following the onset of juvenile idiopathic inflammatory arthritis, in terms of remission of disease activity, loss of function and structural damage based on a review of the available published data. METHODS: Electronic databases were searched for major studies publishing outcome data in the past 10 yr in juvenile idiopathic arthritis, juvenile rheumatoid arthritis and juvenile chronic arthritis, and 21 studies were selected. The proportions of children in the different categories of the outcomes of interest are described. Data were stratified where possible by disease subtype. RESULTS: There were major differences between the studies reviewed in terms of study design, case selection and the results obtained. In general, children with systemic- or polyarticular-onset disease were much less likely to go into remission than those with oligoarticular onset, although the remission rates in the latter group ranged from 36 to 84%. Several different approaches were used to assess functional outcome but the pattern of results between the different subgroups was the same as with remission. Similarly, children with polyarticular disease in all the cohorts reviewed were substantially more likely to have erosive radiological damage on follow-up. The rates of individual outcomes, even within a subgroup, varied considerably between studies and this does not appear to be explained solely by differences in methodology. CONCLUSIONS: There remains a considerable lack of clarity in the prognosis following onset of juvenile idiopathic arthritis for the major outcomes considered, although those with oligoarthritis at presentation have the best outcome. The ability to offer accurate prognosis is particularly important to both reassure parents and guide treatment at disease onset. To achieve this, large definitive prospective studies will be required.     

A specific HLA-DP beta allele is associated with pauciarticular juvenile rheumatoid arthritis but not adult rheumatoid arthritis.

Nonradioactive sequence-specific oligonucleotide probes specific for the HLA-DP beta locus have been used in a simple dot-blot format to type samples amplified by the polymerase chain reaction from 44 patients with pauciarticular juvenile rheumatoid arthritis, 32 patients with adult rheumatoid arthritis, and 50 random controls. The sequences of four new DP beta alleles derived from these patients and controls are reported, bringing the total number of alleles identified thus far to 19. The DPB2.1 allele is significantly increased in juvenile rheumatoid arthritis patients over controls; this allele is not increased in patients with adult rheumatoid arthritis. The association of juvenile rheumatoid arthritis with the DPB2.1 allele is independent of linkage with previously defined HLA-D region markers of disease. Analysis of the DPB2.1 sequence shows that it differs from the nonsusceptible DPB4.2 allele by only 1 amino acid at position 69 in the beta 1 domain.     

Development of an observation method for assessing pain behaviors in children with juvenile rheumatoid arthritis

Objective. To develop an observation method for assessing pain behaviors in children with juvenile rheumatoid arthritis (JRA). Methods. Thirty children with JRA performed a standardized sequence of activities for video recording, and correlations between the pain behaviors observed on the videotapes and established measures of pain, depression, and functional disability were determined. Results. Pain behaviors were reliably observed (kappa coefficients 0.53-0.79). Total pain behaviors were significantly correlated with subjective reports of pain (r = 0.50) and disability levels (r = 0.64). These behaviors were not significantly associated with children's depression ratings (r = 23). Conclusion. The results indicate that the behavioral observation method provides a reliable and valid measure of pain associated with JRA. Measurement of pain behaviors may be especially useful in treatment outcome studies because these behaviors are relatively independent of depression.     

Popliteal cysts in a pediatric patient

A 12-year-old boy with recurrent complaints of knee pain and swelling for six months, but without diagnosed rheumatological disease, presented with calf swelling and knee pain of several days duration. Examination revealed findings consistent with juvenile rheumatoid arthritis and a ruptured Baker's cyst. This is the sixth reported case of ruptured Baker's cyst reported in a child less than 14 years old and should alert emergency physicians that calf pain and swelling secondary to a ruptured popliteal (Baker's) cyst may be an early manifestation of juvenile rheumatoid arthritis.     

Total joint replacement in childhood arthritis

Juvenile rheumatoid arthritis poses a distinct challenge to pediatric rheumatologists and orthopedic surgeons. Recent developments in the medical management of juvenile rheumatoid arthritis have decreased the need for surgical intervention in this subset of patients; however, those patients who need surgery are often the most challenging cases due to their relatively small bone size, the complex deformity caused by soft tissue contractures, and the tendency for multiple joint involvement. Nonetheless, technologic improvements in implant design and surgical techniques have led to successful outcomes in otherwise debilitating conditions. A careful and coordinated approach to the surgical management of juvenile rheumatoid arthritis can lead to improved function and significant pain relief for children with rheumatoid arthritis.     

Therapy of juvenile chronic arthritis with sulfasalazine

Sulfasalazine was used as second line agent in 15 children with different forms of rheumatoid arthritis. In 9 cases amelioration could be achieved with respect to clinical symptoms as well as laboratory parameters. Thus, sulfasalazine can be recommended as basic medication also in juvenile rheumatoid arthritis.     

Juvenile idiopathic arthritis: parent-child discrepancy on reports of pain and disability

OBJECTIVE: To examine the incidence and nature of disagreements about pain and functional disability between parents and their children with juvenile idiopathic arthritis (JIA) and to identify demographic and psychosocial predictors of parent-child disagreement about pain and functional disability. METHODS: Participants comprised 63 children 8-16 years of age (mean 12.36 +/- 2.61) and their parents, followed as part of a longitudinal study of pain in children. During routine rheumatology clinic visits, children and their parents completed validated measures of pain, depressive symptoms, and functional disability. RESULTS: Parents and children often disagreed as to the frequency and intensity of pain and to the degree of disability caused by arthritis. Child depressive symptoms (p < 0.01) and parental perceptions of child limitations (p < 0.02) predicted parent-child disagreement about the frequency of the child's pain. Parental perceptions of child limitations also predicted parent-child disagreement about the child's level of functional disability (p < 0.04). Those children who estimated their level of disability to be different than their parents' rating also were more depressed compared to children who agreed with their parents about their level of disability (p < 0.01). CONCLUSION: Discrepancy between parent and child reports of pain and disability in children with JIA is common. Findings suggest that such disagreements in reporting of pain and functional disability by parents and their children with JIA are associated with underlying depressive symptoms in children.