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Objective: To determine the intra- and interobserver reliability of ultrasound (US)-detected age-related joint vascularization and ossification grading in healthy children., Methods: Following standardized image acquisition and machine setting protocols, 10 international US experts examined 4 joints (wrist, second metacarpophalangeal joint, knee, and ankle) in 12 healthy children (divided into 4 age groups: 2-4, 5-8, 9-12, and 13-16 years). Gray-scale was used to detect the ossification grade, and power Doppler ultrasound (PDUS) was used to detect physiologic vascularization. Ossification was graded from 0 (no ossification) to 3 (complete ossification). A positive PDUS signal was defined as any PDUS signal inside the joint. Kappa statistics were applied for intra- and interobserver reliability., Results: According to the specific joint and age, up to 4 solitary PDUS signals (mean 1.5) were detected within each joint area with predominant localization of the physiologic vascularization in specific anatomic positions: fat pad, epiphysis, physis, and short bone cartilage. The kappa values for ossification grading were 0.87 (range 0.85-0.91) and 0.58 for intra- and interobserver reliability, respectively. The bias-adjusted kappa values for intra- and interobserver reliability were 0.71 (range 0.44-1.00) and 0.69, respectively., Conclusion: Detection of normal findings (i.e., grading of physiologic ossification during skeletal maturation and identification of physiologic vessels) can be highly reliable by using clear definitions and a standardized acquisition protocol. These data will permit development of a reliable and standardized US approach for evaluating pediatric joint pathologies., (© 2019, American College of Rheumatology.)

Background: Recently preliminary ultrasonography (US) definitions, in B mode, for normal components of pediatric joints have been developed by the OMERACT US group. The aim of the current study was to include Doppler findings in the evaluation and definition of normal joint features that can be visualized in healthy children at different age groups., Methods: A multistep approach was used. Firstly, new additional definitions of joint components were proposed during an expert meeting. In the second step, these definitions, along with the preliminary B-mode-US definitions, were tested for feasibility in an exercise in healthy children at different age groups. In the last step, a larger panel of US experts were invited to join a web-based consensus process in order to approve the developed definitions using the Delphi methodology. A Likert scale of 1-5 was used to assess agreement., Results: Physiological vascularity and fat pad tissue were identified and tested as two additional joint components in healthy children. Since physiological vascularity changes over the time in the growing skeleton, the final definition of Doppler findings comprised separate statements instead of a single full definition. A total of seven statements was developed and included in a written Delphi questionnaire to define and validate the new components. The final definitions for fat pad and physiological vascularity agreed by the group of experts reached 92.9% and 100% agreement respectively in a web survey., Conclusion: The inclusion of these two additional joints components which are linked to detection of Doppler signal in pediatric healthy joints will improve the identification of abnormalities in children with joint pathologies.

Objective: The Outcome Measures in Rheumatology Ultrasound Task Force has recently started to work on the validation and standardization of musculoskeletal ultrasound (MSUS) examination in children in order to improve its applicability to joint examination., Methods: This was a prospective multicenter study performed by 4 experts in pediatric MSUS, who independently collected representative images using predefined scanning procedures of 4 joints (knee, ankle, wrist, and second metacarpophalangeal joint) in different predefined age groups. Researchers were allowed to use their own settings (B-mode and Doppler) in order to get the best quality image and highest sensitivity for low blood flow. Images were evaluated for quality parameters and an atlas was created with the best images. An equipment comparative study was performed by a single examiner using 2 different types of machines., Results: Sixty-four healthy children were scanned. The quality of evaluated images, obtained by predefined scanning positions, was highly comparable among the examiners. The B-mode images clearly showed age-related variations of joint findings, while Doppler images showed the presence of blood flow, particularly within the epiphyseal cartilage of the children at a younger age. There was a high to good level of consistency between images obtained from the 2 different ultrasound machines., Conclusion: The study shows a systematic method for ultrasound examination of children at different age groups. Additionally, a baseline collection of images was developed, showing blood vessels in the joints examined. The present study could provide a framework for ongoing MSUS studies as well as for clinical practice in pediatric rheumatology., (© 2016, American College of Rheumatology.)

Objective: To summarize the work performed by the Outcome Measures in Rheumatology (OMERACT) Ultrasound (US) Task Force on the validity of different US measures in rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) presented during the OMERACT 11 Workshop., Methods: The Task Force is an international group aiming to iteratively improve the role of US in arthritis clinical trials. Recently a major focus of the group has been the assessment of responsiveness of a person-level US synovitis score in RA: the US Global Synovitis Score (US-GLOSS) combines synovial hypertrophy and power Doppler signal in a composite score detected at joint level. Work has also commenced examining assessment of tenosynovitis in RA and the role of US in JIA., Results: The US-GLOSS was tested in a large RA cohort treated with biologic therapy. It showed early signs of improvement in synovitis starting at Day 7 and increasing to Month 6, and demonstrated sensitivity to change of the proposed grading. Subsequent voting questions concerning the application of the US-GLOSS were endorsed by > 80% of OMERACT delegates. A standardized US scoring system for detecting and grading severity of RA tenosynovitis and tendon damage has been developed, and acceptable reliability data were presented from a series of exercises. A preliminary consensus definition of US synovitis in pediatric arthritis has been developed and requires further testing., Conclusion: At OMERACT 11, consensus was achieved on the application of the US-GLOSS for evaluating synovitis in RA; and work continues on development of RA tenosynovitis scales as well as in JIA synovitis.

US is a powerful tool for the assessment of joint synovitis in children with JIA and has been shown to be more accurate than clinical examination in detecting synovial disease. Recent studies have documented the presence of US-detected synovial pathology in children with JIA in clinical remission. US assessment enables the differentiation of joint synovitis from tenosynovitis, may help detect enthesitis and is valuable for capturing cartilage damage and early bone erosions. Guidance to local injection therapy represents an important application of US in routine care. Although US has a great potential for diffusion among paediatric rheumatologists, several issues need to be addressed. In particular, a thorough knowledge of US anatomy of joints in growing children is necessary to interpret US findings in JIA patients. The present review examines the potential role of US in the assessment of joint disease in children with JIA.

Imaging assessments of the joints of children with juvenile idiopathic arthritis (JIA) are challenging, owing to the unique features of the growing skeleton. Traditionally, imaging studies in childhood arthritis have been based on conventional radiography. However, in the past few years, interest in the use of MRI and ultrasonography has increased. As a result, imaging has become a main area of clinical and research investigation in paediatric rheumatology. The chief advance in the field of conventional radiography has been the development and validation of paediatric scoring systems for the assessment of radiographic progression. Several studies have shown that MRI provides a precise quantification of synovitis in children with JIA. Furthermore, a high frequency of bone marrow oedema and bone erosions has been found early in the disease course. Ultrasonography has been proven to be superior to clinical examination in detecting synovitis, tenosynovitis and enthesitis. A high frequency of subclinical synovitis has been demonstrated in patients with JIA who have clinically inactive disease using both MRI and ultrasonography. However, more information from healthy children is needed to enable differentiation of the bone and cartilage abnormalities that reflect damage from those that are part of normal development using MRI or ultrasonography. This Review provides a summary of the current information on conventional radiography, ultrasonography and MRI in JIA and highlights the advantages and limitations of each imaging modality.

Objective: To develop and validate a composite disease activity score for juvenile idiopathic arthritis (JIA), the Juvenile Arthritis Disease Activity Score (JADAS)., Methods: The JADAS includes 4 measures: physician global assessment of disease activity, parent/patient global assessment of well-being, active joint count, and erythrocyte sedimentation rate. These variables are part of the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30), Pedi 50, and Pedi 70 criteria for improvement. Validation analyses were conducted on >4,500 patients and included assessment of construct validity, discriminant validity, and responsiveness to change. Three versions of the JADAS were tested based on 71-joint (range 0-101), 27-joint (range 0-57), or 10-joint (range 0-40) counts. Statistical performances of the JADAS were compared with those of 2 rheumatoid arthritis composite scores, the Disease Activity Score in 28 joints (DAS28) and the Clinical Disease Activity Index (CDAI)., Results: The JADAS demonstrated good construct validity, yielding strong correlations with JIA activity measures not included in the score and moderate correlations with the Childhood Health Assessment Questionnaire. Correlations obtained for the 3 JADAS versions were comparable, but superior to those yielded by the DAS28 and CDAI. The area under the curve of the JADAS predicted long-term disease outcome, measured as radiographic progression over 3 years. In 2 clinical trials, the JADAS discriminated well between ACR Pedi 30, Pedi 50, and Pedi 70 response and revealed strong responsiveness to clinical change., Conclusion: The JADAS was found to be a valid instrument for assessment of disease activity in JIA and is potentially applicable in standard clinical care, observational studies, and clinical trials.

Objective: To investigate the influence of arthritis in individual joint groups on subdimensions of functional ability questionnaires in children with juvenile idiopathic arthritis (JIA)., Methods: 206 patients were included who had the Childhood Health Assessment Questionnaire (C-HAQ) and the Juvenile Arthritis Functionality Scale (JAFS) completed simultaneously by a parent and received a detailed joint assessment. In each patient, joint involvement (defined as presence of swelling, pain on motion/tenderness and/or restricted motion) was classified in 3 topographic patterns: Pattern 1 (hip, knee, ankle, subtalar and foot joints); Pattern 2 (wrist and hand joints); Pattern 3 (elbow, shoulder, cervical spine and temporomandibular joints). Frequency of reported disability in each instrument subdimension was evaluated for each joint pattern, present either isolatedly or in mixed form., Results: Among patients with Pattern 1, the JAFS revealed the greatest ability to capture and discriminate functional limitation, whereas impairment in the C-HAQ was more diluted across several subdimensions. Both C-HAQ and JAFS appeared to be less reliable in detecting functional impairment in the hand and wrist (Pattern 2) than in other body areas. Overall, the JAFS revealed a superior ability to discriminate the relative functional impact of impairment in individual joint groups among patients with mixed joint patterns., Conclusion: In children with JIA, a functional measure focused to assess the function of individual joint groups (the JAFS) may detect with greater precision the functional impact of arthritis in specific body areas than does a standard questionnaire based on the assessment of activities of daily living (the C-HAQ).

Objective: To develop and test reduced joint counts in children with juvenile idiopathic arthritis (JIA)., Methods: Four reduced joint counts including 45, 35, 27, and 10 joints were devised by a panel of experienced pediatric rheumatologists, who selected the joints to be included based on the ease of technical assessment, functional relevance, and frequency of involvement. Three large samples of patients with JIA (total n=4353) who had a detailed joint assessment available were used to develop and test reduced joint counts. Performance of reduced counts was examined by comparing their Spearman correlation with the standard (i.e., complete) joint count. Construct validity was evaluated by calculating Spearman correlation with other JIA outcome measures. Responsiveness to clinical change was determined through the standardized response mean (SRM)., Results: Spearman correlations of reduced joint counts with the whole joint count and with the other JIA outcome measures were comparable, revealing that they had similar ability to serve as surrogate for the whole joint count and construct validity. Responsiveness to clinical change was also comparable across reduced counts (SRM 0.83-1.09 for active joint counts and 0.63-0.81 for restricted joint counts). Based on these results and considering the relative feasibility of the different counts, the 27-joint reduced count is proposed for use in JIA. This joint count includes the cervical spine and the elbow, wrist, metacarpophalangeal (from first to third), proximal interphalangeal, hip, knee, and ankle joints., Conclusion: Reduced joint counts appear to be as reliable as standard joint counts in assessment of the severity of joint disease and its change over time in children with JIA.

Objective: To develop and validate a definition of minimal disease activity (MDA) in patients with juvenile idiopathic arthritis (JIA)., Methods: The clinical charts of JIA patients followed over a 16-year period were reviewed to identify visits with high disease activity and MDA, defined on the basis of therapeutic decisions made by the attending physician. For each JIA activity measure recorded at the time of the visit, the cutoff value that best identified states of MDA was calculated by means of the area under the receiver operating characteristic curve analysis. A definition of MDA for oligoarthritis and polyarthritis was set up after testing the relative power of each variable in a multivariate analysis. Validation procedures included assessment of discriminant and construct validity., Results: The definition that resulted from the analyses led to establish that a state of MDA could be defined as the presence of a physician global assessment < or =2.5 cm and a swollen joint count of 0 in patients with oligoarthritis; and as the presence of a physician global assessment < or =3.4 cm, a parent global assessment < or =2.1 cm, and a swollen joint count < or =1 in patients with polyarthritis. Validation procedures demonstrated that the MDA definition had good discriminant and construct validity in the context of both observational studies and controlled trials., Conclusion: We developed a preliminary definition of MDA in patients with JIA that represents a useful treatment target state and is proposed for inclusion as an outcome measure in future observational studies and clinical trials in patients with JIA.

Objective: To describe the longitudinal course of physical functioning in children with juvenile idiopathic arthritis (JIA) and identify predictors of long-term functional impairment., Methods: Between January 1987 and December 2002, 227 patients had two or more functional ability questionnaires completed by a parent. The total number of questionnaires was 1356 and the follow-up between first and last questionnaire administration was 949.7 patient years. At each questionnaire administration, patients were assigned to one of three functional disability states (1 = no disability; 2 = mild to moderate disability; 3 = severe disability), based on their functional ability score. Predictor variables included sex, onset age, JIA category, age at visit, disease duration, presence of antinuclear antibodies, joint counts, acute phase reactants and initial disability state., Results: Despite patient variability in the course of physical functioning, the following three longitudinal patterns were observed: (1) a stable state of disability throughout the entire study period, with continued absence of disability in 27.8% of patients and persistently moderate disability in 3.5% of patients; (2) a steady improvement (22.9% of patients) or deterioration (5.7% of patients) in disability over time; (3) a fluctuating course of disability, with deterioration and improvement (40.1% of patients). Younger age at disease onset and a greater restricted joint count were the strongest predictors of long-term functional impairment., Conclusion: A wide within-patient and between-patient variability in the longitudinal course of functional disability was found. Children with early disease onset and a greater number of restricted joints had the highest risk of developing long-term physical disability.

Objective: To describe the patterns and time course of arthritis in patients with antinuclear antibody (ANA)-positive juvenile idiopathic arthritis (JIA)., Methods: We identified patients followed during a 16-year period who had JIA by ILAR criteria, were ANA-positive (i.e., had >or= 2 positive ANA test results at titer >or= 1:160), and had a disease duration >or= 2 years. Demographic and clinical features, including ILAR category and cumulative number and type of joints affected over time, were recorded., Results: A total of 195 patients were studied. The ILAR category was oligoarthritis in 159 patients and rheumatoid factor-negative polyarthritis in 36 patients. The cumulative rate of polyarticular extension in patients with oligoarticular onset was 26%, 38%, 45%, 49%, and 51% at 1, 2, 3, 4, and 5 years, respectively. At disease onset, most patients had monoarthritis and 95% had

Objective: To develop and validate a clinical measure of articular and extraarticular damage in patients with juvenile idiopathic arthritis (JIA)., Methods: The Juvenile Arthritis Damage Index (JADI), which is derived from physical examination and a brief review of the patient's clinical history, is composed of 2 parts: assessments of articular damage (JADI-A) and extraarticular damage (JADI-E). Instrument validation was accomplished by evaluating 158 JIA patients with disease duration of at least 5 years, seen consecutively over 21 months. The instrument's feasibility, face and content validity, construct and discriminative ability, internal consistency, and interrater reliability were examined., Results: Among the 158 JIA patients, 47% and 37% had articular and extraarticular damage, respectively. The JADI was found to be feasible and to possess both face and content validity. The JADI-A score correlated highly with the number of joints with limited range of motion (Spearman's r [r(S)] = 0.72) and correlated moderately with the Childhood Health Assessment Questionnaire score (r(S) = 0.41), Steinbrocker functional classification (r(S) = 0.50), and Poznanski's score of radiographic damage (r(S) = -0.54), thereby demonstrating good construct validity. Correlations with the JADI-E score were lower, owing to the heterogeneity of its items. The JADI-A discriminated well among different levels of disability. The internal consistency (Chronbach's alpha) of the JADI-A and JADI-E was 0.93 and 0.59, respectively. The intraclass correlation coefficients between pairs of independent observers ranged from 0.85 to 0.97., Conclusion: The JADI exhibited good reliability, construct validity, and discriminative ability and is therefore a valid instrument for the assessment of long-term damage in patients with JIA, in the context of both clinical management and research settings.