title: Preliminary criteria for clinical remission for select categories of juvenile idiopathic arthritis. Reliability of exercise testing and functional activity questionnaires in children with juvenile arthritis. Preliminary definition of disease flare in juvenile rheumatoid arthritis. The relationship between health-related quality of life, pain, and coping strategies in juvenile arthritis – A one year prospective study The Visual Analogue WOMAC 3.0 scale - internal validity and responsiveness of the VAS version Comparisons of Five Health Status Instruments for Orthopedic Evaluation Measuring health status in early juvenile idiopathic arthritis: determinants and responsiveness of the child health questionnaire. Methotrexate improves the health-related quality of life of children with juvenile idiopathic arthritis. Preliminary definition of improvement in juvenile arthritis. Preliminary validation of clinical remission criteria using the OMERACT filter for select categories of juvenile idiopathic arthritis. Clinical responsiveness of self-report functional assessment measures for children with juvenile idiopathic arthritis undergoing intraarticular corticosteroid injections. Development of a digital Childhood Health Assessment Questionnaire for systematic monitoring of disease activity in daily practice.
abstract: OBJECTIVE To develop preliminary criteria for inactive disease and clinical remission for select categories of juvenile idiopathic arthritis ( JIA ) , and to decide what such clinical states should predict in terms of probability of disease recurrence . METHODS A Delphi serial question naire consensus-formation approach was used initially to gather criteria in use by pediatric rheumatologists ( PR ) for defining clinical remission in oligoarticular ( persistent and extended ) , rheumatoid factor ( RF ) positive and negative polyarticular , and systemic JIA . Results from sequential question naires provided an agenda for a nominal group technique ( NGT ) conference to reach consensus on unresolved questions . RESULTS One hundred and thirty PR from 34 countries responded to the question naires and 20 PR from 9 countries attended the conference . Draft criteria for inactive disease include the following : no active arthritis ; no fever , rash , serositis , splenomegaly , or generalized lymphadenopathy attributable to JIA ; no active uveitis ; normal erythrocyte sedimentation rate or C-reactive protein ; and a physician 's global assessment of disease activity rated at the best score possible for the instrument used . According to consensus vote , 6 continuous months of inactive disease on medication defines clinical remission on medication , while 12 months of inactive disease off all anti-arthritis ( and anti-uveitis ) medications defines clinical remission off medication . The finalized criteria for remission off medication ideally should predict that a patient has < /= 20 % probability of disease recurrence within the next 5 years . CONCLUSION Using consensus formation techniques , we formulated preliminary criteria for inactive disease and clinical remission on and off medication for use in select categories of JIA . Retrospective validation is in progress ; prospect i ve validation will follow . Future efforts will include other categories of JIA OBJECTIVE To determine the reliability of formal exercise testing and the reliability of functional and activity question naires in children with juvenile idiopathic arthritis ( JIA ) . METHODS Children with JIA of any subtype ages 8 - 16 years who were recruited to a r and omized trial comparing different exercise therapies participated in 2 preintervention sessions of exercise testing 2 - 6 weeks apart . Exercise testing included 1 ) submaximal oxygen uptake ( VO(2submax ) ) , 2 ) peak VO(2 ) ( VO(2peak ) ) , and 3 ) anaerobic power using modified Wingate tests ( W(ant ) ) . Two physical function question naires ( the Childhood Health Assessment Question naire [ C-HAQ ] and Revised Activity Scale for Kids [ ASK ] ) and 1 daily physical activity question naire ( the Habitual Activity Estimation Scale [ HAES ] ) were also completed at these times . Test-retest reliability was assessed using type 3 , intrarater intraclass correlation coefficient ( ICC(3,1 ) ) and Bl and and Altman plots were used to determine limits of agreement . RESULTS Data were available for 74 patients ( 58 girls ) .  VO(2submax ) , VO(2peak ) , and W(ant ) demonstrated high reliability ( ICC(3,1 ) 0.82 , 0.91 , and 0.94 , respectively ) . C-HAQ and ASK question naires also had very high reliability ( ICC(3,1 ) 0.82 and 0.91 , respectively ) . The HAES demonstrated low reliability for total activity score ( ICC(3,1 ) 0.15 ) and moderate reliability when the number of very active hours was analyzed separately ( ICC(3,1 ) 0.59 ) . CONCLUSION Results of this investigation suggest that exercise testing and functional question naires in children with JIA are consistent and reliable . Reliability of the HAES total score was poor , but moderate when the very active hours subscale score was used OBJECTIVE To develop preliminary criteria for defining disease flare in patients with polyarticular-course juvenile rheumatoid arthritis ( JRA ) . METHODS Data from a r and omized clinical trial of etanercept in JRA ( 51 patients ) and the 6 core response variables ( CRV ) for JRA were used to derive flare definitions . The criterion st and ard of flare was treatment with placebo . C and i date flare definitions were assessed by receiver-operator characteristic ( ROC ) curve properties and other statistics for diagnostic tests . RESULTS Of the possible flare definitions tested with acceptable statistical properties , the one that seemed to be the most useful was worsening in any 2/6 CRV by > or = 40 % without improvement in more than 1 of the remaining CRV by > or = 30 % . Two other superior flare definitions were ( 1 ) worsening in 3/6 CRV by > or = 30 % and ( 2 ) any worsening of the Childhood Health Assessment Question naire , worsening of erythrocyte sedimentation rate by > or = 30 % and worsening of the active joint count by > or = 10 % . CONCLUSIONS CRV are useful for defining flare in JRA . Worsening in any 2/6 CRV by > or = 40 % without concomitant improvement of more than one of the remaining CRV by > or = 30 % appears to be the most suitable preliminary flare definition . Because the proposed flare criteria were derived from a small number of patients , it is essential to perform more definitive testing of this and several alternative flare definitions in larger patient population The aim of this 12-month prospect i ve study was to compare reports describing the health-related quality of life ( HRQL ) of children with Juvenile idiopathic arthritis ( JIA ) obtained from parents and children , to investigate the extent to which the children ’s HRQL changed over a 12-month period , and to describe the relationship between children ’s HRQL , and their experience of pain and use of pain coping strategies . Fifty-four children aged 8–18 years with JIA and their parents completed st and ard question naires assessing children ’s HRQL , pain intensity , and pain coping strategies at baseline , 6 months , and 12 months . In general , children reported that their HRQL was better than was reported by parents . Both informants described children ’s HRQL as being very stable over the 12 months of the study . Consistent with the Biobehavioural Model of Pain , there was a significant negative relationship between children ’s HRQL and their experience of pain . However , there was little evidence that pain coping strategies mediated the relationship between children ’s experience of pain and their HRQL Background Many people suffer with Osteoarthritis ( OA ) and subsequent morbidity . Therefore , measuring outcome associated with OA is important . The Western Ontario and McMaster Universities Osteoarthritis Index ( WOMAC ) has been a widely used patient reported outcome in OA . However , there is relatively little evidence to support the use of the Visual Analogue Scale ( VAS ) version of the scale . We aim ed to explore the internal validity and responsiveness of this VAS version of the WOMAC . Methods Patients with chronic hip or knee pain of mechanical origin , waiting for a hip or knee joint replacement completed the WOMAC as part of a study to investigate the effects of acupuncture and placebo controls . Validity was tested using factor analysis and Rasch analysis , and responsiveness using st and ardised response means . Results Two hundred and twenty one patients ( mean age 66.8 , SD 8.29 , 58 % female ) were recruited . Factor and Rasch analysis confirmed unidimensional Pain and Physical Functioning scales , capable of transformation to interval scaling and invariant over time . Some Differential Item Functioning ( DIF ) was observed , but this cancelled out at the test level . The Stiffness scale fitted the Rasch model but adjustments for DIF could not be made due to the shortness of the scale . Using the interval transformed data , St and ardised Response Means were smaller than when using the raw , ordinal data . Conclusions The WOMAC Pain and Physical Functioning subscales satisfied unidimensionality and ordinal scaling tests , and the ability to transform to an interval scale . Some Differential Item Functioning was observed , but this cancelled out at the test level and , by doing so , at the same time removed the disturbance of unidimensionality . The scaling characteristics of sets of items which use VAS require further analysis , as it would appear that they can lead to spurious levels of responsiveness and scale compression because they exaggerate the distortion of the ordinal scale . Trial numberUKCRN study ID : 4881IS RCT This study represents a long-term effort to find optimal techniques for evaluating outcome in patients who have undergone total joint arthroplasty . Sensitivity of five health status question naires was studied in a longitudinal evaluation of orthopedic surgery . The question naires ( Arthritis Impact Measurement Scales [ AIMS ] , Functional Status Index [ FSI ] ,  Health  Assessment  Question naire [ HAQ ] , Index of  Well  Being [ IWB ] , and Sickness Impact Profile  [ SIP ] ) were administered to 38 patients with end-stage arthritis at three points in time : two weeks before hip or knee arthroplasty , and at three-month and 12- to 15-month follow-up . Response values ( i.e. , changes within patients ) were calculated on four scales : global health , pain , mobility , and social function . By the three-month follow-up , most instruments detected large mean responses in global health , pain scores , and mobility . Smaller changes on these scales were found between three and 12 to 15 months . Social function showed small to modest gains at successive follow-ups . St and ardized response means were calculated to assess sensitivity to detect change . Confidence intervals for these indices were constructed using a jackknife procedure , and significance tests were performed by pairing selected indices . Finally , the study projected sample sizes required to assess a new therapy , using each response . These statistical tools facilitated comparisons among instruments and may prove useful in other setting OBJECTIVE To assess the determinants and responsiveness of the Norwegian version of the Child Health Question naire ( CHQ ) in patients with early juvenile idiopathic arthritis ( JIA ) and to compare health status in patients and controls . METHODS A total of 116 children ( median age 8.4 yrs ) with JIA and < 2.5 years of disease duration ( median 11.0 mo ) were examined by a pediatric rheumatologist and reassessed after a median of 10.0 months . Physical and psychosocial health were assessed by means of the CHQ , which provides summary scores for physical and psychosocial health , the Childhood Health Assessment Question naire ( CHAQ ) , and the Child Behavior Checklist ( CBCL , n = 32 ) . Matched controls ( n = 116 ) , r and omly selected from the general population , completed the CHQ at baseline . RESULTS The patients with JIA had poorer physical health and slightly impaired psychosocial health compared with the controls [ 41.2 + /- 13.6 vs 55.2 + /- 7.3 ( p < 0.001 ) and 51.0 + /- 7.5 vs 54.1 + /- 5.7 ( p = 0.002 ) , respectively ] . The most important determinants of the CHQ physical summary score were the child 's pain , morning stiffness , the CHAQ disability index , erythrocyte sedimentation rate ( ESR ) , overall well-being , and physician 's global assessment of disease activity . The psychosocial summary score correlated with the CBCL level of internalizing , externalizing , and total behavior problems . The st and ardized response mean for the physical summary score was large ( 0.96 ) for those who improved , and moderate ( -0.60 ) for those who became worse . CONCLUSION The CHQ discriminated between patients with early JIA and controls . The most important determinants of the CHQ physical summary score were the child 's pain , morning stiffness , CHAQ , ESR , overall well-being , and physician 's global assessment of disease activity . The CHQ was sensitive to clinical changes in children with JIA OBJECTIVES To examine the change in health-related quality of life ( HRQOL ) and its determinants in children with juvenile idiopathic arthritis ( JIA ) treated with methotrexate ( MTX ) .  METHODS Patients were extracted from the PRINTO clinical trial which aim ed to evaluate the efficacy and safety profile of MTX administered in st and ard , intermediate or higher doses ( 10 , 15 and 30 mg/m(2)/week respectively ) . Children with polyarticular-course JIA , who were less than 18 years and had a complete HRQOL assessment were included . RESULTS A total of 521 children were included . At baseline , patients with JIA showed poorer HRQOL ( p<0.01 ) than healthy children . In 207/412 ( 50 % ) and 63 ( 15 % ) children , HRQOL values were 2 st and ard deviations below the mean of healthy controls in the physical and psychosocial summary scale , respectively . After 6 months of treatment with st and ard dose MTX , there was a statistically significant improvement in all HRQOL health concepts , particularly the physical ones . Similar improvements were observed in those who did not respond to a st and ard dose of MTX and were subsequently r and omised to a higher dose . The presence of marked disability at baseline was associated with a fivefold increased risk of retaining poor physical health after 6 months of active treatment with st and ard dose MTX . Other less important determinants of retaining poor physical well-being were the baseline level of systemic inflammation , pain intensity and an antinuclear-antibody-negative status . CONCLUSIONS MTX treatment produces a significant improvement across a wide range of HRQOL components , particularly in the physical domains , in patients with JIA OBJECTIVE To identify a core set of outcome variables for the assessment of children with juvenile arthritis ( JA ) , to use the core set to develop a definition of improvement to determine whether individual patients demonstrate clinical ly important improvement , and to promote this definition as a single efficacy measure in JA clinical trials . METHODS A core set of outcome variables was established using a combination of statistical and consensus formation techniques . Variables in the core set consisted of 1 ) physician global assessment of disease activity ; 2 ) parent/patient assessment of overall well-being ; 3 ) functional ability ; 4 ) number of joints with active arthritis ; 5 ) number of joints with limited range of motion ; and 6 ) erythrocyte sedimentation rate . To establish a definition of improvement using this core set , 21 pediatric rheumatologists from 14 countries met , and , using consensus formation techniques , scored each of 72 patient profiles as improved or not improved . Using the physicians ' consensus as the gold st and ard , the chi-square , sensitivity , and specificity were calculated for each of 240 possible definitions of improvement . Definitions with sensitivity or specificity of < 80 % were eliminated . The ability of the remaining definitions to discriminate between the effects of active agent and those of placebo , using actual trial data , was then observed . Each definition was also ranked for face validity , and the sum of the ranks was then multiplied by the kappa statistic . RESULTS The definition of improvement with the highest final score was as follows : at least 30 % improvement from baseline in 3 of any 6 variables in the core set , with no more than 1 of the remaining variables worsening by > 30 % . The second highest scoring definition was closely related to the first ; the third highest was similar to the Paulus criteria used in adult rheumatoid arthritis trials , except with different variables . This indicates convergent validity of the process used . CONCLUSION We propose a definition of improvement for JA . Use of a uniform definition will help st and ardize the conduct and reporting of clinical trials , and should help practitioners decide if a child with JA has responded adequately to therapy . We are in the process of prospect ively validating this definition and several others that scored highly OBJECTIVE To begin the validation process of the preliminary criteria for inactive disease ( ID ) , clinical remission on medication ( CRM ) , and clinical remission off medication ( CR ) in children with select forms of juvenile idiopathic arthritis ( JIA ) . METHODS We used the OMERACT filter paradigm to estimate the validity of the criteria within each of the filter 's 3 components : truth , discrimination , and feasibility , in 5 categories of JIA : systemic arthritis , persistent and extended oligoarthritis , and rheumatoid factor-positive and negative polyarthritis . Data sources for determining validity estimates included a Delphi question naire survey sent to 246 pediatric rheumatologists in 34 countries , a consensus conference attended by 20 senior pediatric rheumatologists representing 9 countries , a retrospective chart review of 437 patients with JIA from 3 tertiary care clinics who had been followed between 4 and 22 years , and the literature . RESULTS Truth component : face and content validity . These aspects of validity were largely established via the Delphi question naire exercise and the consensus conference . Using an 80 % consensus level , participants felt that a set of non-redundant variables could effectively differentiate the clinical states of ID , CRM , and CR . Criterion validity could not be irrefutably established because no gold st and ard for inactive disease exists for JIA . As an alternative , published investigations of remission in JIA were used to estimate concurrent and convergent validity , as surrogates for criterion validity and as indicators of overall construct validity . Correlational analyses revealed the new criteria to have good construct validity . Discrimination component : the criteria demonstrated moderate to high levels of classification , prognosis , and responsiveness ( sensitivity to change ) using data from the chart review . Patients who were able to attain CR remained disease-free for substantially longer periods than did those who attained only ID or CRM . Responsiveness was evidence d by the ability of the criteria to allow movement of most patients between the disease states , consistent with what is known of the course of the disease . Feasibility component : Results of the Delphi and consensus conference produced a set of criteria that are easily , quickly , and inexpensively completed in the physician 's office , and present minimal or no risk to the patient . CONCLUSION The preliminary criteria demonstrated moderate to excellent validity characteristics in some , but not all components of the OMERACT filter . Prospect i ve validation studies are under way OBJECTIVE The Childhood Health Assessment Question naire ( CHAQ ) , Juvenile Arthritis Functional Assessment Report ( JAFAR ) , and Juvenile Arthritis Functional Status Index ( JASI ) are widely used functional measures for juvenile idiopathic arthritis ( JIA ) that differ in content , format , and completion time . We compared the responsiveness and child-parent agreement of the JAFAR , CHAQ , and JASI in a prospect i ve , multicenter study . METHODS Children and adolescents from 5 rheumatology centers were enrolled . Subjects were about to undergo therapy ( intraarticular corticosteroid injections [ IAS ] and methotrexate or hip surgery ( MTX/hip ] ) expected to produce a functional improvement . All subjects were studied before the intervention and at 6 weeks and 6 months posttreatment . At each study visit , the 3 measures were administered in r and omized , balanced order to both parents and children . RESULTS A total of 92 subjects ( mean age 12.8 years ) were enrolled in the study , 74 of which were in the IAS group . The responsiveness of all 3 measures was moderate to strong . The st and ardized response mean at 6 weeks for the IAS group on the JAFAR , CHAQ , and JASI was 0.41 ( 95 % confidence interval [ 95 % CI ] 0.18 , 0.64 ) , 0.70 ( 95 % CI 0.47 , 0.93 ) , and 0.36 ( 95 % CI 0.13 , 0.59 ) , respectively . The CHAQ was somewhat more responsive to change at 6 weeks ( IAS group : relative efficiency 0.34 [ JAFAR ] , 0.27 [ JASI ] ) , but less responsive at 6 months ( MTX/hip group : relative efficiency 5.1 [ JAFAR ] , 3.9 [ JASI ] ) . All 3 question naires showed acceptable parent-child agreement , and overall , there were few differences between the 3 question naires . CONCLUSION The functional outcome measures currently used for JIA are all adequately responsive for use in trials or in the clinic setting . The choice of which measure to use should therefore be based on the time available for completion , the intended clinical / research use , and the depth of content required OBJECTIVE To develop a reliable and user-friendly digital Childhood HAQ ( CHAQ ) to facilitate systematic monitoring of disease activity at the outpatient clinic in juvenile idiopathic arthritis ( JIA ) patients . METHODS The digital CHAQ was tested with patients who visited the outpatient paediatric rheumatology clinic of the Erasmus MC Sophia Children 's Hospital . These patients completed in a r and omized order the paper form and digital CHAQ while being observed . Validity was tested by comparing outcomes with the paper form CHAQ .  User-friendliness was evaluated through a short question naire . RESULTS A digital CHAQ was developed and revised several times according to our observations . Outcome is automatically calculated and can be printed . Fifty-one patients completed both the digital and paper form CHAQ . Correlation coefficient between both outcomes of the CHAQ Disability Index was 0.974 . No statistically significantly differences in median outcome were found in visual analogue scale ( VAS ) pain ( 25.6 vs 25.9 mm ) and VAS well-being ( 20.1 vs 19.5 mm ) . Although the mean time ( 5.06 min ) to complete the digital CHAQ was significantly longer than the mean time ( 3.75 min ) to complete the paper form , the majority of patients ( 75 % ) preferred the digital version . User-friendliness received maximum positive score . CONCLUSION We developed a reliable and user-friendly digital CHAQ , which can be easily and systematic ally completed during routine clinic visits . Such digitalization of question naires can be applied in any field to make systematic monitoring of disease activity in daily practice possible
target: The  Childhood Health Assessment Question naire ( C-HAQ )  was the most extensively studied instrument and had high reliability , but only moderate correlations with other indices of disease activity and poor responsiveness to change in disease status .
  The physician global assessment of disease activity ( PGA ) and articular indices had the strongest association with disease activity and were the most responsive to change .
  Measures of psychosocial function and quality of life were moderately associated with measures of disease activity , but were less responsive to changes in disease status .
  In children with JIA , no single instrument was superior in reliability or validity or in describing the impact of JIA .
  Although the C-HAQ has been extensively evaluated , the PGA and articular indices appear to have the highest responsiveness to change and , therefore , the highest potential for detecting important differences in treatment response
background: OBJECTIVE To review the performance characteristics of the instruments most commonly used to measure clinical outcomes in juvenile idiopathic arthritis ( JIA ) , including global assessment s , articular indices , functional/disability assessment s , and quality of life measures .
  METHODS As part of an Agency for Healthcare Research and Quality comparative effectiveness review of antirheumatic drugs , we explored the characteristics of commonly used outcome measures for JIA .