Juvenile idiopathic arthritis and future risk for cardiovascular disease: a multicenter study.

OBJECTIVES: To evaluate the frequency of cardiovascular disease (CVD) and CVD risk factor development in adult patients previously diagnosed with juvenile idiopathic arthritis (JIA). METHOD: A cohort study was conducted utilizing patients at two academic institutions (cohorts 1 and 2). Each institution evaluated the common endpoint of CVD outcomes and CVD risk factor development in adults aged ≥ 30 years and at the 29-year follow-up from disease onset in cohorts 1 and 2, respectively, with comparison to control groups of similar age and sex. RESULTS: Cohort 1 included 41 patients with JIA and follow-up ≥ 30 years of age with comparison to 41 controls. Three patients (7%) had CVD, compared to one control (2%; p = 0.31). Cohort 2 included 170 patients with JIA and a median of 29 years of follow-up from disease onset with comparison to 91 controls. Two patients (2%) had CVD, compared to none of the controls (p = 0.29). The presence of CVD risk factors was found to be increased in the JIA group compared to the controls in three categories: family history of CVD (cohort 1), hypertension (cohort 2), and ever smokers (cohorts 2). CONCLUSIONS: There is no increase in CVD events in patients with JIA 29 years following disease onset when compared to the general population. As these cohorts age, it will be informative to evaluate whether this baseline risk remains present or a trend towards increasing CVD emerges. Continued longitudinal follow-up of these cohorts and larger population-based studies are needed to establish a definitive relationship between JIA and CVD.

The association of PTPN22 rs2476601 with juvenile idiopathic arthritis is specific to females.

A preponderance of females develop autoimmune disease, including juvenile idiopathic arthritis (JIA), yet the reason for this bias remains elusive. Evidence suggests that genetic risk of disease may be influenced by sex. PTPN22 rs2476601 is associated with JIA and numerous other autoimmune diseases, and has been reported to show female-specific association with type 1 diabetes. We performed main effect and sex-stratified association analyses to determine whether a sex-specific association exists in JIA. As expected, rs2476601 was associated with JIA in our discovery (413 cases and 690 controls) and replication (1008 cases and 9284 controls) samples. Discovery sample sex-stratified analyses demonstrated an association specifically in females (odds ratio (OR)=2.35, 95% confidence interval (CI)=1.52-3.63, P=0.00011) but not males (OR=0.91, 95% CI=0.52-1.60, P=0.75). This was similarly observed in the replication sample. There was evidence for genotype-by-sex interaction (Pinteraction=0.009). The association between rs2476601 and JIA appears restricted to females, partly accounting for the predominance of females with this disease.

CIITA gene variants are associated with rheumatoid arthritis in Scandinavian populations.

Expression of the major autoimmune risk loci DRB1 and DQB1 is regulated by the class II MHC (major histocompatibility complex) transactivator (CIITA), making the CIITA gene a strong autoimmune risk locus candidate. A CIITA promoter single-nucleotide polymorphism (SNP), rs3087456 (-168 A/G), has indeed been associated with several autoimmune diseases, including rheumatoid arthritis (RA). Recently, an intronic SNP rs8048002 has been suggested as a better susceptibility marker in Addison's disease. Therefore, we tested both SNPs in a panel of autoimmune diseases, consisting of Norwegian patients with RA (n=819), juvenile idiopathic arthritis (JIA; n=524), or type 1 diabetes (T1D; n=1211), and 2149 controls. We also included an independent Swedish RA cohort (n=2503) and controls (n=1416). Both rs3087456 and rs8048002 were significantly associated with RA (combined Norwegian and Swedish patients P(corrected)=0.012 and P(corrected)=0.0016, respectively), but not with JIA or T1D. Meta-analysis of 16 RA cohorts confirmed rs3087456 with only marginal significance (P=0.016). However, results were stronger in the Scandinavian subgroup (4 cohorts, P=3.8 × 10(-4)), indicating a population-dependent effect. A similar pattern was observed in a meta-analysis of rs8048002. Our results support involvement of CIITA in RA, but imply that this is population dependent and that the aetiological variant is yet to be discovered.

Self-rated physical and psychosocial health in a cohort of young adults with juvenile idiopathic arthritis.

OBJECTIVE: To explore self-rated physical and psychosocial health in a cohort of young adults with juvenile idiopathic arthritis (JIA) 18.3 years after symptom onset, make comparisons with population-based data, and illuminate possible predictors of self-rated health. METHODS: Of a baseline cohort of 84 patients with JIA, 55 (65.5%) answered the self-administered questionnaires of the Health Assessment Questionnaire (HAQ), the Visual Analogue Scale (VAS) of pain, fatigue, and illness, the Medical Outcomes Study 36-item Short Form Health Survey (SF-36), and the General Health Questionnaire (GHQ-30). Telephone interviews were conducted with 51/55 patients. Population-based norm-data of SF-36 were used for comparison. RESULTS: Significantly impaired physical health but no difference in psychosocial health was found as compared to the general Norwegian population. The level of education was significantly higher whereas no difference was found in employment status as compared to norm-data. Pain was a significant correlate of the education level. Predictors of physical impairment were physical disability and pain, whereas psychiatric distress and female sex were predictors of mental ill-health. CONCLUSION: Physical disability does not seem to have a negative influence on the patients' functioning psychosocially.

Craniofacial growth disturbance is related to temporomandibular joint abnormality in patients with juvenile idiopathic arthritis, but normal facial profile was also found at the 27-year follow-up.

OBJECTIVES: To assess the long-term outcome of craniofacial morphology related to disease variables and temporomandibular joint (TMJ) involvement as demonstrated with computed tomography (CT) and magnetic resonance imaging (MRI) in adult patients with juvenile idiopathic arthritis (JIA). METHODS: Sixty of 103 patients participated in a re-examination on average 27 years after baseline. Craniofacial morphology, with emphasis on size and position of the mandible, was assessed in lateral cephalographic images and related to disease variables and TMJ involvement by CT and MRI. Definitions of craniofacial growth disturbances were based on measurements outside 2 SD from the mean of healthy adult controls. RESULTS: Sagittal craniofacial growth disturbances were found in 57% and micrognathia in 27% of the 60 patients. Of those with JIA TMJ involvement, 70% had some form of growth disturbance. Micrognathia occurred only in patients with bilateral TMJ involvement. The bilateral TMJ group had significantly different craniofacial morphology than healthy controls and patients without TMJ involvement. Growth disturbances and TMJ involvement were present in all subtypes of JIA, except for one subtype comprising one patient. Patients with growth disturbances had more severe disease than patients with normal craniofacial growth, regarding both present and previous disease activity. Unexpectedly, half of the patients without craniofacial growth disturbances also had TMJ involvement, many from before the age of 12. CONCLUSIONS: Craniofacial growth disturbances were found to be frequent in adult JIA patients, especially in those with bilateral TMJ involvement. However, growth disturbances did not always follow TMJ involvement, not even when affected early.

The efficacy of rehabilitation for patients with rheumatoid arthritis: comparison between a 4-week rehabilitation programme in a warm and cold climate.

OBJECTIVES: To investigate the long-term effect (week 16) of a 4-week rehabilitation programme for patients with rheumatoid arthritis (RA) and to compare the effect of this intervention given in a Mediterranean or a Norwegian climate. METHODS: A randomized, controlled, parallel group design, where 124 RA patients applying for rehabilitation were randomized to a rehabilitation programme either in Norway or in a Mediterranean climate. The participants were examined clinically immediately before (week 0) and after (week 4) the rehabilitation period as well as in week 16 and answered a mailed questionnaire in week 28. The 28-Joint Disease Activity Score (DAS28), American College of Rheumatology (ACR) response and physical tests were used to measure clinical response. RESULTS: The baseline DAS28 value 4.45 (1.16) was reduced by -0.95 (1.05) in the Mediterranean climate and the baseline DAS28 value 4.18 (1.17) was reduced by -0.37 (0.92) in the Norwegian climate at week 16 (p = 0.003). An ACR20 improvement was achieved in 25% of the patients treated in the Mediterranean climate and in 15% of those treated in the Norwegian climate. Sustained improvement in all ACR core components at week 16 and in patient's assessment of health status at week 28 was found in the patients treated in the Mediterranean climate only. Tests of physical function, the 6-Minute Walk Test (6MWT) and the Timed Up and Go (TUG), showed comparable improvements in patients treated in both climates. CONCLUSIONS: RA patients showed immediate positive effects with regard to disease activity, physical function, and symptoms during a 4-week rehabilitation programme. The effects on disease activity and symptoms were larger and better maintained at least 3 months after rehabilitation in a warm rather than in a cold climate.

A longitudinal follow-up study of physical and psychosocial health in young adults with chronic childhood arthritis.

OBJECTIVE: The aim was to describe physical and psychosocial health status in a second follow-up of a cohort of patients with chronic childhood arthritis, to compare results from the present study with the first follow-up, and to explore the course of physical and psychosocial functioning from baseline. METHODS: At a median of 18.3 years after symptom onset 55 patients answered the self-administered questionnaires Health Assessment Questionnaire Disability Index (HAQ-DI), Visual Analogue Scales (VAS) of pain, fatigue and illness, and General Health Questionnaire (GHQ) 30-item version. Results from the current study were compared to first follow-up median 8.7 years after symptom onset, and the course of physical and psychosocial function from baseline was discussed. RESULTS: At second follow-up, 38% reported HAQ-DI above 0 indicating physical disability, 22% had a GHQ-30 score in the clinical range indicating psychiatric distress, and fatigue seemed to be an overarching aspect of the health status. Pain was an important correlate of physical disability at first and second follow-up. At second follow-up psychiatric distress was a significant correlate of pain and fatigue, indicating a relation to disease severity. The association between psychosocial functioning and chronic family difficulties observed at first follow-up is not evident at second follow-up. CONCLUSIONS: The favourable physical and psychosocial outcome reported at first follow-up seems to persist. However, arthritis-related ill-health is still evident in a considerable proportion of the patients, indicating a constant impact of the disease on every-day life of the individual.

The FCRL3 -169T>C polymorphism is associated with rheumatoid arthritis and shows suggestive evidence of involvement with juvenile idiopathic arthritis in a Scandinavian panel of autoimmune diseases.

BACKGROUND AND OBJECTIVES: The Fc receptor-like 3 (FCRL3) gene -169T>C single nucleotide polymorphism (SNP) has been reported to be associated with several autoimmune diseases (AIDs) in Japanese populations. However, association results in other populations have been conflicting. Therefore, we investigated this SNP in a Scandinavian panel of AIDs. METHODS: We genotyped patients with rheumatoid arthritis (RA; n = 708), juvenile idiopathic arthritis (JIA; n = 524), systemic lupus erythaematosus (SLE; n = 166), ulcerative colitis (UC; n = 335), primary sclerosing cholangitis (PSC; n = 365), Crohn disease (CD; n = 149), a healthy control group (n = 1030) and 425 trio families with type 1 diabetes (T1D). Statistical analysis consisted of case-control and family-based association tests. RESULTS: RA was associated with the C allele (odds ratio (OR) = 1.16, 95% CI 1.01 to 1.33) and the CC genotype (OR = 1.30, 95% CI 1.01 to 1.67) of the FCRL3 -169T>C SNP in our material. Suggestive evidence for association was also found for JIA (CC genotype: OR = 1.30, 95% CI 0.99 to 1.70), and clinical subgroup analysis indicated that this was connected to the polyarticular subgroup. No significant association was found with SLE, UC, CD, PSC or T1D. In patients with RA, we found no significant interaction between the FCRL3 -169T>C and PTPN22 1858C>T SNPs, nor between the FCRL3 -169CC genotype and IgM-rheumatoid factor or anti-cyclic citrullinated peptide titre levels. CONCLUSION: We found an association between the FCRL3 -169T>C SNP and RA, and suggestive evidence for involvement with JIA, in a Norwegian population. These findings lend support for a role for this SNP in RA across ethnically diverse populations, and warrant follow-up studies in JIA.

Recent-onset childhood arthritis--association with Streptococcus pyogenes in a population-based study.

OBJECTIVES: To assess the frequency of Streptococcus pyogenes in children with early arthritis, compare the characteristics in patients with post-streptococcal ReA (PSReA) with those in patients with other types of arthritis, and describe the occurrence of carditis in PSRA. PATIENTS: In a population-based Norwegian study, the physicians were asked to refer all children with suspected arthritis. The arthritis patients were followed up at 6 weeks, 6 months and 18 months. The presence of S. pyogenes was based on throat smear or antibodies. Echocardiography was performed in the patients with ARF or PSRA. RESULTS: Thirty-two (18%) of the 173 children with arthritis tested positive for S. pyogenes. The percentage of positive tests rose steadily with age and peaked at ages 8-11 (35%). Six weeks after admission arthritis was present in 33% of the PSRA patients, which was less frequent than in the juvenile idiopathic arthritis (JIA) patients (P < 0.001), but more frequent than in the transient arthritis patients (P = 0.012). Hip arthritis was more frequent and knee/ankle arthritis, ANA and HLA-B27 were less frequent in PSRA than in JIA (P < 0.001, P = 0.009 and P = 0.029, respectively). The PSRA patients were older than those with transient arthritis (P = 0.007). One child with ARF had carditis. CONCLUSIONS: Streptococcus pyogenes was present in 18% of children with arthritis. The patient characteristics, clinical presentation and early disease course in PSRA was different from that of JIA and transient arthritis.

Disease duration, hypertension and medication requirements are associated with organ damage in childhood-onset systemic lupus erythematosus.

OBJECTIVE: To investigate the frequency of organ damage in childhood-onset systemic lupus erythematosus (SLE) and to identify disease variables and patient characteristics related to organ damage. METHODS: A cohort of 71 patients was examined in a cross-sectional study after a mean disease duration of 10.8+/-8.2 years (mean age 26.4+/-9.8 years). The occurrence of organ damage was measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Factors analysed as possible explanatory variables of organ damage were the following: demographic variables, clinical variables at diagnosis and during disease course, as well as medication use. Growth and self-reported health status were also measured. RESULTS: The most frequent areas of organ damage were in the neuropsychiatric (28%), renal (13%) and musculoskeletal (13%) organ systems. Forty-three patients (61%) had evidence of damage. The mean SDI score was 1.3 for the whole study population. Hypertension, longer disease duration and use of cyclophosphamide were factors significantly related to an increasing SDI score in multiple linear regression analyses. Furthermore, patients with damage (SDI > or =1) compared to those without damage (SDI = 0) had a significantly higher cumulative corticosteroid dose (24.7 g versus 10.6 g) and more frequently required high-dose prednisolone at diagnosis (68% versus 43%). CONCLUSION: Evidence of organ damage was found in 61% of all patients. Long disease duration, known hypertension and use of cylophosphamide were significantly associated with an increasing SDI score. Furthermore high-dose prednisolone at diagnosis and cumulative prednisolone dose were significantly related to the presence of organ damage.

Association to HLA-DRB1*08, HLA-DPB1*0301 and homozygosity for an HLA-linked proteasome gene in juvenile ankylosing spondylitis.

To assess the role of HLA genes other than those encoding B27 in predisposing to JAS and AAS, we analyzed the distribution of B*4001, as well as the DRB1, DPB1, and LMP2 alleles, using PCR-based techniques in 63 JAS and 44 AAS patients (all B27 positive). The NBMDR (N = 4724) provided a source of controls matched with the patients for B27 (or other markers when necessary). We found an increase of the B*4001, DRB1*08, and DPB1*0301 alleles, as well as the LMP2 b/b genotype (the latter was most pronounced among patients with acute iridocyclitis), in JAS compared to B27-positive controls. The increase of DRB1*08 and DPB1*0301 was due to an increase of DRB1*08 and DPB1*0301 in combination, whereas the association with B*4001 could be due to linkage disequilibrium with LMP2b. None of these associations were detected in AAS. We conclude that in JAS, in addition to the association to B27, there are also weaker but distinct associations to the DRB1*08, DPB1*0301 alleles and homozygosity for LMP2b.

Interaction between HLA-DR and HLA-DP, and between HLA and interleukin 1 alpha in juvenile rheumatoid arthritis indicates heterogeneity of pathogenic mechanisms of the disease.

EOP-JRA is an autoimmune disease that displays associations with DPB1*0201, DR8, DR5, and DR6, as well as an association with IL1A2 (a variant of IL1 alpha gene, not HLA linked). The purpose of this study was to analyze interactions between these genetic factors. We studied 103 EOP-JRA patients, 181 random controls, and 69 DR8-positive controls. We found a positive interaction between DPB1*0201 and the DRB1 alleles encoding DR3, DR5, or DR6, but not DR8. In addition, we found evidence for an interaction between IL1A2 and DR(3, 5, or 6) and DP2, but not DR8. We interpret the data to suggest heterogeneity in the HLA-associated pathogenic mechanisms of EOP-JRA.

Psychosocial outcome in juvenile chronic arthritis: a nine-year follow-up.

OBJECTIVE: To describe the long-term psychosocial outcome in a prospectively followed cohort of patients with juvenile chronic arthritis (JCA), to assess the associations between psychosocial outcome and disease variables and to explore family stressors as predictors of long-term psychosocial and physical outcome. METHODS: Fifty-two patients with JCA were assessed psychosocially at first admission to a pediatric rheumatology clinic and were reassessed 9 years later. Assessment methods included semi-structured psychiatric interviews and standardized parental questionnaires and self-reports. RESULTS: At follow-up, 9 patients (17%) fulfilled the criteria for a psychiatric diagnosis and 8 (15%) had mild to moderate impairment in psychosocial functioning (children's or adult Global Assessment Scale). Mental health and psychosocial functioning were significantly improved from the first hospital admission to follow-up. In patients < 18 years of age (n = 26), psychosocial functioning at follow-up correlated with physical disability according to the Childhood Health Assessment Questionnaire (r = -0.52, p < 0.01). Psychosocial outcome was unrelated to other measures of disease severity. Chronic family difficulties in the disease course predicted psychosocial functioning at follow-up in patients < 18 years old (R2 = 0.22). Chronic family difficulties at disease onset, together with gender and chronic family difficulties in the disease course, predicted psychosocial functioning at follow-up in patients > or = 18 years old (R2 = 0.61). Family stressors were unrelated to the physical outcome. CONCLUSION: The long-term psychosocial outcome was favorable in most of the patients. Psychosocial outcome was predicted by chronic family difficulties, but was not closely related to disease severity at follow-up.

Outcome and predictive factors in children with chronic idiopathic musculoskeletal pain.

OBJECTIVE: The aim of the present study was to describe the outcome and determine predictors of persisting chronic idiopathic musculoskeletal pain in children. METHODS: A prospective 9-year follow-up of 37 children with musculoskeletal pain of at least 3 months duration for which no physical origin could be found, was carried out. The study comprised those patients with idiopathic pain in a cohort of 117 first admissions to a pediatric rheumatology clinic; 72 patients with juvenile chronic arthritis (JCA) were used as a comparison group. RESULTS: Twenty-two patients (59%) still had chronic idiopathic musculoskeletal pain at the 9-year follow-up, while 15 patients no longer had pain after a median of 2.1 years (range 0.3-8.9). Compared with the patients with resolved pain, those with chronic pain had a longer disease duration before admission (median 1.4 versus 0.5 years, P < 0.01), more frequent generalised pain (86 versus 47%, P < 0.05), more intense pain (median 4.3 versus 0.5 cm VAS, P < 0.05), a lower parental education level (mean 10 versus 14 years, P < 0.01) and more chronic family difficulties (mean score 4.3 versus 2.9, P < 0.01) on first admission. Predictors of persistent pain were generalised pain on first admission (OR = 84) and a low mother's education level (OR = 0.31 per year of increased education). At follow-up, 16 patients (73%) with persistent chronic pain reported some disability according to the childhood or the adult Health Assessment Questionnaire (CHAQ/HAQ). The patients with chronic pain had as high a pain intensity (median 2.7 versus 2.0 cm VAS, NS), as much disability (median CHAQ/HAQ 0.3 versus 0.3) and as much impact on overall well-being (median 2.9 versus 3.2 cm VAS, NS) as patients with active JCA, but they had more fatigue (median 5.1 versus 1.3 cm VAS, P < 0.05), lower levels of psychosocial functioning (median score 74 versus 80, P < 0.05) and more chronic family difficulties (median score 3.3 versus 2.3, P < 0.001) than the JCA patients. CONCLUSION: Chronic idiopathic musculoskeletal pain in children had an unfavourable outcome in the present study, especially in children with generalised pain and a low parental education level.

The Norwegian version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report herein the results of the cross-cultural adaptation and validation into the Norwegian language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Norwegian CHAQ and CHQ have already been published and therefore they were fully revalidated in this study. A total of 148 subjects were enrolled: 88 patients with JIA (6% systemic onset, 45% polyarticular onset, 10% extended oligoarticular subtype, and 39% persistent oligoarticular subtype) and 60 healthy children. The CHAQ clinically discriminated between patients with various JIA subtypes, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to those with persistent oligoarticular arthritis. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Norwegian version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

Toxicity of antirheumatic and anti-inflammatory drugs in children.

The aim of the study was to describe the long-term toxicity of antirheumatic and anti-inflammatory drugs in a paediatric rheumatology clinic population. One hundred and seventeen children were studied on first admission to a paediatric rheumatology clinic and after a mean of 8.6 +/- 0.4 years of follow-up. Medical records from the intermediate period were reviewed. The patients had 155 exposures to non-steroidal anti-inflammatory drugs (NSAIDs), 88 exposures to disease-modifying antirheumatic drugs (DMARDs) and 12 exposures of prednisolone during a total of 682 patient years. Drug toxicity was measured in terms of the number of toxic events, number of drug discontinuations due to toxicity, number of side-effects per patient year of drug exposure and as a toxicity index. Side-effects were seen in 69 (27%) of the drug exposures, corresponding to 0.10 toxic events per patient year of exposure. Abdominal pain was the most common side-effect, and was reported in 21 (14%) of the exposures to NSAIDs. Five severely toxic events, all leading to hospitalisation, occurred. The toxicity of NSAIDs was not significantly different from that of DMARDs with regard to the number of toxic events (21% and 31%, respectively, NS) and drug discontinuations due to toxicity (17% and 14%, respectively, NS). Piroxicam tended to be more toxic than ibuprofen (46% versus 18% toxic events, p <0.05; 36% versus 16% discontinuations due to toxicity, NS; 0.33 versus 0.05 side-effects per patient year and a toxicity index of 1.45 versus 0.20 units per patient year). Gold tended to be more toxic than antimalarials (41% versus 15% toxic events, p<0.05; 24% versus 12% discontinuations, NS; 0.37 versus 0.08 side-effects per patient year and a toxicity index of 1.56 versus 0.23 units per patient year). It was concluded that antirheumatic and anti-inflammatory drugs led to side-effects in 27% of the exposed children during 9 years of follow-up. There was an overlap of the toxicity of certain NSAIDs and the most commonly employed DMARDs.

Relationship between disease course in the temporomandibular joints and mandibular growth rotation in patients with juvenile idiopathic arthritis followed from childhood to adulthood.

OBJECTIVE: To investigate the relationship between radiographic JIA disease course in the TMJs and mandibular growth rotation, compared with growth in healthy individuals. METHODS: From a larger series of JIA patients followed from childhood to adulthood, 26 were included; 11 without and 15 with bilateral radiographic TMJ involvement. Joint morphology and function were assessed at baseline, 2-, 4-, 6- and 27 years follow-up. Mandibular growth rotation (anterior, posterior or none) was assessed from cephalometric evaluations at childhood and adulthood, with observations from 16 healthy individuals as controls. TMJ disease course and mandibular growth rotation were assessed independently and their relationship analysed. Non-parametric statistical methods were applied to test differences between groups. RESULTS: In the normal TMJ group of JIA patients the joint morphology was similar at the follow-ups and all patients had good function both in childhood and in adulthood. The mandibular growth rotation was similar to that of healthy controls, i.e. predominantly in anterior direction. In the abnormal TMJ group different JIA TMJ disease courses were observed and associated with changes in the mandibular growth rotation (p = 0.007).Progressing JIA TMJ disease course was related to posterior mandibular growth rotation and improving disease course to anterior mandibular growth rotation. CONCLUSION: A relationship was found between JIA disease course in the TMJs and mandibular growth rotation, suggesting that a favourable growth could be regained in patients with improvement in TMJ morphology and/or TMJ function. To confirm this, further research on larger patient series is needed.

Health-related quality of life of patients with juvenile idiopathic arthritis coming from 3 different geographic areas. The PRINTO multinational quality of life cohort study.

OBJECTIVES: To compare health-related quality of life (HRQL) and to identify clinical determinants for poor HRQL of patients with juvenile idiopathic arthritis (JIA) coming from three geographic areas. METHODS: The HRQL was assessed through the Child Health Questionnaire (CHQ). A total of 30 countries were included grouped in three geographic areas: 16 countries in Western Europe; 10 in Eastern Europe; and four in Latin America. Potential determinants of poor HRQL included demographic data, physician's and parent's global assessments, measures of joint inflammation, disability as measured by Childhood Health Assessment Questionnaire (CHAQ) and erythrocyte sedimentation rate. Poor HRQL was defined as a CHQ physical summary score (PhS) or psychosocial summary score (PsS) <2 S.D. from that of healthy children. RESULTS: A total of 3167 patients with JIA, younger than 18 yrs, were included in this study. The most affected health concepts (<2 S.D. from healthy children) that differentiate the three geographic areas include physical functioning, bodily pain/discomfort, global health, general health perception, change in health with respect to the previous year, self-esteem and family cohesion. Determinants for poor HRQL were similar across geographic areas with physical well-being mostly affected by the level of disability while the psychosocial well-being by the intensity of pain. CONCLUSION: We found that patients with JIA have a significant impairment of their HRQL compared with healthy peers, particularly in the physical domain. Disability and pain are the most important determinants of physical and psychosocial well-being irrespective of the geographic area of origin.