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.     

Brief report: adjustment to juvenile rheumatoid arthritis: a family systems perspective

OBJECTIVE: To examine the relations of the family environment to adjustment to juvenile rheumatoid arthritis (JRA), and to examine how those relations are influenced by child sex and age. METHOD: Ninety-four children with JRA completed a questionnaire on family environment and adjustment. RESULTS: Family cohesion was related to good adjustment, whereas family conflict was related to poor adjustment. Some relations of family cohesion to adjustment were stronger for younger than for older children. The relations of child autonomy to adjustment depended on child sex and age. CONCLUSION: The relations of the family environment to adjustment to JRA are dependent on child sex and age.     

Psychosocial factors in juvenile diabetes: A review

Studies assessing (1) the influence of psychosocial factors on the onset of juvenile diabetes, (2) the influence of psychosocial factors on the course of this disease, and (3) the influence of diabetes on the psychosocial development of the child are reviewed. Directions for future research are suggested.     

Psychosocial adjustment in children with kidney disease

Objective: To examine family environment, levels of parentingstress, and child behavior problems in children with one ofthree kidney diseases compared to healthy children and to examinepredictors of psychological distress in the full sample. Method: Parents of children with steroid sensitive nephroticsyndrome, chronic renal insufficiency, or kidney transplant (n = 41) were compared to 34 healthy children of similar demographic characteristics. Results: Mean scores on family functioning, parenting stress,and child behavior were within normal limits. Family environment variables significantly predicted child behavior and parentingstress for parents of ill and healthy children. Qualitativeresponses provided insight into developmentally specific stressorsand intervention needs in the illness groups. Conclusions: These data indicate that long-term survivors ofkidney disease function similarly to demographically matchedpeers and that the family environment may buffer stress causedby illness. Specific concerns raised by parents in the kidneydisease groups indicate the need to appropriately assess andintervene with this understudied population.     

Psychological adjustment of children and adolescents with chronic arthritis: a meta-analytic review

OBJECTIVE: To review studies of psychological adjustment among children and adolescents with chronic arthritis to determine whether they are at more risk for development of adjustment problems than controls. METHODS: We used meta-analytic techniques to review 21 studies reporting overall adjustment problems, internalizing symptoms, externalizing symptoms, or self-concept among youths with arthritis. RESULTS: Youths with arthritis displayed increased risk for overall adjustment problems and internalizing symptoms, but not for externalizing symptoms or poor self-concept. Risk was greater in studies making comparisons to study controls rather than to norms and in studies including mixed disease samples (arthritis plus other rheumatic diseases) rather than samples of youths with arthritis only. CONCLUSIONS: Results suggest the importance of assessing for internalizing problems among youths with chronic arthritis. Future research may benefit from inclusion of child self-report of adjustment problems, diagnostic specificity in reporting results, and use of adjustment measures without somatic items.     

HLA region microsatellite polymorphisms in juvenile arthritis

Abstract: A number of microsatellite polymorphisms located in the MHC region of the human chromosome 6 have been analysed in a large group of patients with juvenile arthritis (JA) ( n =177) and in 157 controls. There have been no significant associations for the alleles of the microsatellite polymorphisms D6S -105, D6S -510, TNFA, TNFC, TNFD, TNFE, HSP. Allele frequencies and HLA associations were listed for the non-associated micro-satellite loci. The microsatellite locus DQ CAR, which is localized between DQA1 and DQB1, shows a significant positive association with JA for the allele DQ CAR 121 and a negative association for the allele DQ CAR 111. The allele DQ CAR 121 is strongly associated with DQA1*0501 and with DQB1*0301 both in the normal controls and in the patient population. This pair of DQA/DQB alleles corresponds to the DQ molecule DQ7 on the cell surface, which has been described to be strongly associated with JA. Investigations of the two and three-point haplotypes of DQ CAR with alleles of its neighboring loci have shown that the association with DQ CAR 121 is secondary to the association with DQ7 previously observed. Thus, using eight HLA linked microsatellite polymorphisms in the region from HLA-A to HLA-DQ, we have not found any evidence for further loci which might be involved in the coding for susceptibility for JA.     

Psychosocial adjustment in males with Duchenne muscular dystrophy: psychometric properties and clinical utility of a parent-report questionnaire

Objective The primary aim of this study was to establish thepsychometric properties and clinical utility of the PersonalAdjustment and Role Skills Scale (PARS-III) for assessing psychosocialadjustment in males with Duchenne muscular dystrophy (DMD).Methods The parents of 287 male patients with DMD aged 5–18years completed the PARS-III and Revised Rutter Scale. ResultsThe coefficients and factor analysis indicated good reliabilityand validity. Overall psychosocial adjustment was not significantlydifferent in DMD compared to males with other chronic medicalconditions and was positively associated with increases in age.A clinical cutoff score for screening in the DMD populationis also reported. Conclusions The PARS-III is a reliable andvalid index of youth psychosocial adjustment in DMD and canbe used for both clinical screening and research purposes.     

Comparing parental distress,family functioning,and the role of social support for caregivers with and without a child with juvenile rheumatoid arthritis

OBJECTIVE: To assess parental distress, family functioning, and social support among parents of children with a lifetime diagnosis of juvenile rheumatoid arthritis (JRA) and comparison families. METHODS: Parents of 64 children with JRA (64 mothers, 46 fathers) completed questionnaires and in-home interviews along with 64 matched comparison families. Average time since diagnosis for children with JRA was 70 months. RESULTS: Families of children with JRA generally reported levels of parental distress, family functioning, and social support similar to those for comparison families. More mothers of children with JRA exceeded the clinical cutoff on the SCL-90-R than comparison mothers. Although disease characteristics and social support did not distinguish subgroups of parents at greater risk for problems, family supportiveness and conflict were associated with caseness for mothers of children with JRA. CONCLUSIONS: Families of children with JRA exhibited substantial resilience over the long term. Further multisite study of children recently diagnosed and with more severe forms of JRA is warranted to determine intervention needs, especially for mothers.     

Autoantibodies to DNA topoisomerase II in juvenile rheumatoid arthritis.

Sera from 58 children with juvenile rheumatoid arthritis were examined for the presence of antibodies to DNA topoisomerase II. Eight sera were reactive in immunoblotting with purified human topoisomerase II and a protein encoded by a cloned cDNA expressed in Escherichia coli which represents the carboxy-terminal domain of the human enzyme. In addition, the sera detect topoisomerase II in mitotic chromosomes and chromosome scaffolds. Five of the sera bind to the native enzyme in solution and deplete such solutions of the active enzyme. All eight sera also contain antibodies to nuclear antigens other than topoisomerase II.     

Interactions between children with juvenile rheumatoid arthritis and their mothers

OBJECTIVE: To determine the degree to which mothers of children with juvenile rheumatoid arthritis (JRA) show an overprotective or highly controlling interaction style. METHOD: We videotaped 84 mother-child pairs (42 JRA and 42 healthy, ages 6 to 13) while working on a collaborative problem-solving task. Based on physical therapy evaluations, children in the JRA group were assigned to "more severe" (n = 19) and "milder" (n = 22) arthritis subgroups. RESULTS: Results showed numerous differences between mothers of children with more severe arthritis and the other mothers (no differences between the milder arthritis and healthy comparison groups were found). Compared to mothers in the other two groups, mothers of children with more severe arthritis were more directive of their children's behavior during the task, showing higher rates of structure and rule setting, general clues, and prompting the child for an answer. DISCUSSION: Sequential analyses showed that mothers in the more severe group appeared to treat the task in a more evaluative manner, being more likely than other mothers to respond to correct answers with positive feedback and to incorrect answers with structure and rule setting. Mothers in the other groups were more likely to respond to both correct and incorrect answers with specific clues. CONCLUSIONS: We discuss how these differences in interactional style might impact the social development of children with JRA.     

Psychosocial adjustment during pregnancy for older couples conceiving through assisted reproductive technology

BACKGROUND: The trend to older maternal age at first birth is well established in Western countries and biological risk factors, particularly declining fertility, are well documented. Less is known, however, about the psychosocial well-being of older first time parents. This study explores differences in psychosocial adjustment during pregnancy in older (maternal age >or= 38 years) and younger (maternal age < 35 years) couples after assisted reproductive technology (ART) conception. METHODS: Questionnaire data were collected from a consecutive cohort of pregnant nulliparous women and their partners recruited over a 12-month period from ART clinics in Sydney, Australia. RESULTS: There were more similarities than differences when comparing older and younger couples. Older couples took longer to conceive and were more likely to use donor eggs. Older pregnant women scored higher on a measure of psychological hardiness/resilience and reported a lower identification with motherhood compared with younger pregnant women. Older men differed only in reporting a less satisfying social orientation during pregnancy (lower satisfaction with sex life, relationship with partner and social life). CONCLUSIONS: Findings do not indicate problematic adjustment during pregnancy in older couples, but differences found need further investigation using larger samples and prospective designs.     

Risk and resistance factors in the adaptation in mothers of children with juvenile rheumatoid arthritis

OBJECTIVE: To examine the importance of illness severity, child functional status, psychosocial stress, intrapersonal factors, stress processing, and social-ecological factors in predicting psychological symptoms among mothers of children with juvenile rheumatoid arthritis (JRA). METHODS: Mothers of 92 children with JRA completed surveys while waiting with their children for physician appointments or during JRA meeting breaks. RESULTS: Mothers reported higher mean levels of psychological symptoms than a normative group. Higher levels of psychosocial stress predicted increased psychological symptoms after accounting for disease severity and functional status. Maternal appraisal of the illness tended to moderate the relationship between illness stress and psychological symptoms, and maternal education moderated the relationship between daily hassles stress and psychological symptoms. CONCLUSIONS: These data indicate that mothers of children with JRA are at risk for psychological distress. Inteventions that take into account the buffering effects of maternal education and appraisal may serve to decrease the effects of maternal stress.     

A matched comparison of adjustment in children with cystic fibrosis and psychiatrically referred and nonreferred children

Utilized the Child Assessment Schedule, a standardized clinical interview, to assess psychosocial adjustment in terms of DSM-III diagnoses. The criteria for a major DSM-III diagnosis were met by 58% of the children with cystic fibrosis compared to 23% for the nonreferred children and 77% for the psychiatrically referred children. Children with cystic fibrosis generally did not demonstrate more symptoms of psychological disturbance than nonreferred children. It was in terms of worries, poor self-image, and anxiety, that the children with cystic fibrosis demonstrated symptom levels comparable to that of psychiatrically referred children. Findings are discussed in terms of the specific risk of internalizing behavior problems for children with chronic illness.     

The impact on health-related quality of life from non-steroidal anti-inflammatory drugs, methotrexate, or steroids in treatment for juvenile idiopathic arthritis

OBJECTIVE: To assess and compare the impact of medication treatments on health-related quality of life (HRQOL), family function, and medical status in children with juvenile idiopathic arthritis (JIA). METHODS: Fifty-seven children diagnosed with JIA were assessed by a pediatric rheumatologist and placed into one of three treatment groups: (1) non-steroidal anti-inflammatory; (2) methotrexate; or (3) steroids via IV methylprednisolone. Questionnaires were administered at baseline and 4-month follow-up. The attending pediatric rheumatologist provided additional medical information. RESULTS: Data document the impact of JIA on HRQOL, particularly on physical and pain domains. Steroid patients experienced improved HRQOL at follow-up relative to other groups, despite reporting more problems with side effects. CONCLUSION: These results demonstrate positive benefits of steroids in treating JIA children, despite the greatest incidence of adverse side effects.     

Association of the HLA-G 14-bp insertion/deletion polymorphism with juvenile idiopathic arthritis and rheumatoid arthritis

We tested the possible association of the 14-bp polymorphism of the HLA-G gene in the course of two inflammatory diseases, rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA). Patients and controls were genotyped for the 14-bp polymorphism by polymerase chain reaction with specific primers for the exon 8 of the human leukocyte antigen (HLA)-G gene and the amplified fragment was visualized in a 6% polyacrylamide gel. A total of 106 JIA patients, 265 RA patients, 356 healthy adults and 85 healthy children were genotyped for the 14-bp polymorphism. Female JIA patients presented a higher frequency of the -14 bp allele when compared with female healthy children (0.743 and 0.500, corrected P=0.003), which reflected in the JIA group as a whole. This increased frequency of the -14-bp allele was observed in all JIA subtypes. In RA patients, no differences in allelic and genotypic frequencies were observed between patients and controls. No correlations were observed among genotype and disease severity or clinical manifestations. Our data suggest that the HLA-G -14 bp allele is probably a risk factor for JIA, mainly in females. Considering the differences observed in relation to gender, we suggest that hormonal differences can interfere with the development of JIA. Considering the RA patients, our data agree with results from the literature and highlight the differences in the etiology of RA and JIA.     

Brief report: use of complementary and alternative medicine and psychological functioning in Latino children with juvenile idiopathic arthritis or arthralgia

OBJECTIVE: To describe the use of complementary and alternative medicine (CAM) and its relationship to symptoms of anxiety, depression, and dysthymia in Latino children with juvenile idiopathic arthritis (JIA) or arthralgia. METHODS: Parents of 36 children between the ages of 6 and 16 years with either JIA (n = 17) or arthralgia (n = 19) completed questionnaires during routine pediatric rheumatology clinic visits assessing use of CAM and psychological functioning. RESULTS: CAM was used by the majority of children primarily to treat pain episodes. The most common modalities were prayer and massage therapy. CAM use was associated with decreased symptoms of anxiety and dysthymia in children with arthralgia, but not in children with JIA. CONCLUSION: Preliminary findings suggest that CAM use is associated with improved psychological functioning in children with arthralgia. Healthcare providers are encouraged to routinely screen for CAM usage and to educate families about the potential benefits and limitations of CAM.     

Predicting uveitis in juvenile idiopathic arthritis: from biomarkers to clinical practice

Introduction: Uveitis is a sight-threatening eye inflammation and common manifestation of juvenile idiopathic arthritis (JIA). New biomarkers that can predict uveitis are needed to alleviate personalized clinical screening. In this review, we outline clinical and molecular risk factors for uveitis and discuss their putative biology and value for clinical practice.

Areas covered: The recent discovery of the YST-amino acid motif in the Human Leukocyte Antigen DRB1 gene exposed a strong genetic predisposition for uveitis in females and can be used to identify low-risk cases and redefine screening policies. The established predictor ‘young age at arthritis onset’ appeared to only hold true for females, emphasizing the importance of sex-stratification in biomarker applications. Aqueous humor profiling studies have shown unique mediator changes. Finally, erythrocyte sedimentation rate and S100A12 levels can be used to stratify patients at high risk for uveitis.

Expert commentary: Various markers have been identified and may significantly improve risk assessment for uveitis in JIA. However, there remains an unmet need to better predict uveitis in advance. Here, we propose a set of markers with high potential for prospective studies, which subsequently can be integrated to develop optimal prediction tools that complement improved screening guidelines for early disease detection and personalized care strategies.     

Intermediate monocytes are increased in enthesitis‐related arthritis,a category of juvenile idiopathic arthritis

Microarray of peripheral blood (PB) and synovial fluid mononuclear cells (PBMC, SFMC) of patients with juvenile idiopathic arthritis–enthesitis‐related arthritis (JIA‐ERA) has shown the involvement of monocytes. On the basis of CD14 and CD16 expression, monocytes are classified as classical, intermediate and non‐classical. In response to Toll‐like receptor (TLR) stimulation, intermediate monocytes produce proinflammatory cytokines and play a role in inflammatory diseases. Therefore, we have studied the microarray profile of monocytes, the frequency of their subsets and cytokine production. Monocyte‐specific microarray analysis was performed in six healthy controls' PBMC and six patients' PBMC and SFMC using Illumina chips WG12. Monocyte subsets were assessed in 46 patients with JIA‐ERA and 17 healthy controls and 17 disease controls by flow cytometry. Interleukin (IL)?23 and tumour necrosis factor (TNF) levels were measured in culture supernatants of eight controls and seven patients' PBMC/SFMC with/without lipopolysaccharide (LPS) stimulation. Cytokine‐producing intermediate monocytes were assessed by flow cytometry. Genes related to antigen presentation, cytokine signalling and TLR pathway were regulated differentially in PB and synovial monocytes of patients with JIA‐ERA. Key genes of intermediate monocytes, such as CLEC10A and MARCO, were expressed three‐ to fourfold more in JIA‐ERA. In PB, the frequency of intermediate monocytes was significantly higher in JIA‐ERA (4·90% ± 3·5) compared to controls (1·8% ± 1·06; P < 0·001). Patients' synovial cells also had more intermediate monocytes compared to PB (11·25% ± 11·32, 5·9% ± 4·8; P = 0.004). Intermediate monocytes are the major producers of IL‐23. Thus, intermediate monocytes may play an important role in JIA‐ERA, possibly by producing cytokines, and contribute to joint inflammation.     

Effect of probiotics on clinical and immune parameters in enthesitis‐related arthritis category of juvenile idiopathic arthritis

Gut microflora and dysbiosis as an environmental factor has been linked to the pathogenesis of enthesitis‐related arthritis (JIA‐ERA); thus, we performed a proof‐of‐concept study of probiotics to modulate the gut‐flora and study the effects on immune and clinical parameters of children having JIA‐ERA. Forty‐six children with active JIA‐ERA were randomized to placebo or probiotic therapy along with non‐steroidal anti‐inflammatory drugs (NSAIDs) for 12 weeks. Patients were assessed using a six‐point composite disease activity index (mJSpADA) based on morning stiffness, joint count, enthesitis count, sacroiliitis/inflammatory back pain, uveitis and erythrocyte sedimentation rate/C‐reactive protein (ESR/CRP). Frequencies of T helper type 1 (Th1), Th2, Th17 and regulatory T cells in blood were measured using flow cytometry. Serum cytokines interferon (IFN)‐γ, interleukin (IL)?4, IL‐17, IL‐10, tumour necrosis factor (TNF)‐α and IL‐6 were measured by cytokine bead array using flow cytometer. The average age of 46 children (44 boys) was 15 ± 2.5 years and duration of disease was 3.5 ± 3 years. There was no significant difference in improvement in mJSpADA between the two groups (P = 0·16). Serum IL‐6 levels showed a decrease (P < 0·05) in the probiotic‐group. Th2 cell frequency (P < 0·05) and serum IL‐10 levels (P < 0·01) showed an increase in the placebo group, but again the probiotic use did not show a significant change in immune parameters when compared to the placebo. Adverse effects among the probiotic and placebo groups were diarrhea (36 versus 45%), abdominal pain (9 versus 20%), minor infections (4·5 versus 20%) and flatulence (23 versus 15%), respectively. Thus, we can conclude that probiotic therapy in JIA‐ERA children is well tolerated, but failed to show any significant immune or clinical effects over NSAID therapy.