Child and Caregiver Beliefs of Importance of Physical Function and Quality of Life in Juvenile Idiopathic Arthritis: A Survey Study.

PURPOSE: To evaluate patient-caregiver beliefs of relative importance across 4 domains while living with juvenile idiopathic arthritis (JIA). METHODS: This was a cross-sectional, anonymous survey study conducted in an academic medical center. Participants with JIA and caregivers (N = 151) completed a Likert-style survey to rate items by importance of knowledge about 4 domains: medications, physical activity, routine measures, and quality of life. RESULTS: Knowledge of medication issues ranked higher than the remaining 3 domains (4.2 ± 0.7 points vs 4.0 ± 0.7, 4.1 ± 0.8, and 4.0 ± 0.9 points, respectively; P = .026; P = .026). Compared with caregivers, participants rated importance lower for all 4 domains. CONCLUSIONS: Gait and physical activity and well-being are not uniformly measured as part of routine clinical care and disease tracking in JIA. Both participants and caregivers ranked knowledge of physical activity similarly to routine office measures and quality of life. Inclusion of these measures in routine care could improve people centeredness and inform treatment plans.

Comprehensive and reliable sonographic assessment and scoring system for inflammatory lesions of the paediatric ankle.

OBJECTIVE: The clinical decision-making process in paediatric arthritis lacks an objective, reliable bedside imaging tool. The aim of this study was to develop a US scanning protocol and assess the reliability of B-mode and Doppler scoring systems for inflammatory lesions of the paediatric ankle. METHODS: As part of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) US group, 19 paediatric rheumatologists through a comprehensive literature review developed a set of standardized views and scoring systems to assess inflammatory lesions of the synovial recesses as well as tendons of the paediatric ankle. Three rounds of scoring of still images were followed by one practical exercise. Agreement among raters was assessed using two-way single score intraclass correlation coefficients (ICC). RESULTS: Of the 37 initially identified views to assess the presence of ankle synovitis and tenosynovitis, nine views were chosen for each B-mode and Doppler mode semi-quantitative evaluation. Several scoring exercises and iterative modifications resulted in a final highly reliable scoring system: anterior tibiotalar joint ICC: 0.93 (95% CI 0.92, 0.94), talonavicular joint ICC: 0.86 (95% CI 0.81, 0.90), subtalar joint ICC: 0.91 (95% CI 0.88, 0.93) and tendons ICC: 0.96 (95% CI 0.95, 0.97). CONCLUSION: A comprehensive and reliable paediatric ankle US scanning protocol and scoring system for the assessment of synovitis and tenosynovitis were successfully developed. Further validation of this scoring system may allow its use as an outcome measure for both clinical and research applications.

Reliability of the Pediatric Specific Musculoskeletal Ultrasound Scoring Systems for the Elbow, Wrist, and Finger Joints.

OBJECTIVE: Musculoskeletal ultrasound (MSUS) is increasingly being used in the evaluation of pediatric musculoskeletal diseases. In order to provide objective assessments of arthritis, reliable MSUS scoring systems are needed. Recently, joint-specific scoring systems for arthritis of the pediatric elbow, wrist, and finger joints were proposed by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) MSUS workgroup. This study aimed to assess the reliability of these scoring systems when used by sonographers with different levels of expertise. METHODS: Members of the CARRA MSUS workgroup attended training sessions for scoring the elbow, wrist, and finger. Subsequently, scoring exercises of B mode and power Doppler (PD) mode still images for each joint were performed. Interreader reliability was determined using 2-way single-score intraclass correlation coefficients (ICCs) for synovitis and Cohen [Formula: see text] for tenosynovitis. RESULTS: Seventeen pediatric rheumatologists with different levels of MSUS expertise (1-15 yrs) completed a 2-hour training session and calibration exercise for each joint. Excellent reliability (ICC > 0.75) was found after the first scoring exercise for all the finger and elbow views evaluated on B mode and PD mode, and for all of the wrist views on B mode. After a second training session and a scoring exercise, the wrist PD mode views reached excellent reliability as well. CONCLUSION: The preliminary CARRA MSUS scoring systems for assessing arthritis of the pediatric elbow, wrist, and finger joints demonstrate excellent reliability among pediatric MSUS sonographers with different levels of expertise. With further validation, this reliable joint-specific scoring system could serve as a clinical tool and scientific outcome measure.

Normal Magnetic Resonance Imaging Appearance of Marrow Adjacent to the Sacroiliac Joints in Children During Development.

OBJECTIVE: On magnetic resonance imaging (MRI) for sacroiliitis, increased T2 marrow signal can be misinterpreted as marrow edema. We hypothesize that a changing but predictable pattern for marrow signal intensity adjacent to the sacroiliac joints is present from infancy through skeletal maturity. The purpose of our study is to characterize the distribution of increased T2 signal intensity within the marrow adjacent to the sacroiliac joints in healthy children. SUBJECTS AND METHODS: A retrospective review of the electronic health record identified 345 children who underwent MRI examination of the sacrum, sacroiliac joints, or pelvis. Those with underlying disease that may potentially alter sacroiliac marrow signal were excluded. Sixty children, 30 girls and 30 boys, were assessed for T2 marrow signal intensity greater than the interforaminal sacrum and less than or equivalent to the primary spongiosa of the posterior iliac crests at the S1, S2, and S3 levels. The width of increased T2 signal intensity at each sacral level, right and left sides, ilium, and sacrum was measured (mm). The Mann-Whitney U test was used to determine if there were differences between skeletally immature and skeletally mature subjects; defined as aged 15 years for girls and 17 years for boys. RESULTS: When 30 girls and 30 boys are assessed, the width of increased T2 marrow signal adjacent to the sacroiliac joints is significantly greater in skeletally immature than in skeletal mature female subjects for right S1 sacral side (P = 0.0100), left S1 sacral side (P = 0.0119), right S2 sacral side (P = 0.037), left S2 sacral side (P = 0.0020), and in male subjects for the right S2 sacral side (P = 0.0178), right S2 iliac side (P = 0.0415), right S3 sacral side (P = 0.0024), and left S3 sacral side (P = 0.0037). There were insufficient subjects for whom the ilii extended beyond the S2 sacral segments to assess for the S3 iliac sides. CONCLUSIONS: Healthy children and adolescents have increased T2 signal intensity within the sacral marrow adjacent to the sacroiliac joints, likely the vascular primary spongiosum, which is greater in adolescence compared with skeletal maturity. Knowledge of this normal pattern is beneficial in interpreting MRI examinations for the presence of sacroiliitis.

Chronic Recurrent Multifocal Osteomyelitis Involving the Spine, Sternum, and Lower Extremities: A Case Report.

Chronic recurrent multifocal osteomyelitis (CRMO) or chronic nonbacterial osteitis is a sterile autoinflammatory disease of bone in children that can mimic infectious osteomyelitis and osteosarcoma. Early diagnosis, treatment, and long-term follow-up of CRMO are essential. We describe a 10-year-old boy who presented with 15 days of left ankle bone more than joint pain, swelling, and limp. Plain radiographs and magnetic resonance imaging scans were nondiagnostic of osteomyelitis and tibial irrigation and biopsy were negative for infection and malignancy. Four years later, he again presented with similar pain in his right ankle. Repeat bone biopsy noted reactive bone changes and bone culture was sterile. Whole-body magnetic resonance imaging revealed multiple enhancing lesions in the long bones of bilateral lower extremities, spine, and sternum. He was diagnosed with CRMO, and treatment with celecoxib and subsequently pamidronate, infliximab, and methotrexate were initiated. After 6 months of treatment, the patient's gait and pain improved, and 2 years later, his CRMO was in clinical and radiologic remission. Of note, he developed palmoplantar pustular psoriasis, commonly seen in CRMO, that was not determined to be from tumor necrosis factor inhibition.

Assessing Pediatric Rheumatology Fellow Competence in the Milestone Era: Past, Present, and Future.

Starting in 2015, pediatric rheumatology fellowship training programs were required by the Accreditation Council for Graduate Medical Education to assess fellows' academic performance within 21 subcompetencies falling under six competency domains. Each subcompetency had four or five milestone levels describing developmental progression of knowledge and skill acquisition. Milestones were standardized across all pediatric subspecialties. As part of the Milestones 2.0 revision project, the Accreditation Council for Graduate Medical Education convened a workgroup in 2022 to write pediatric rheumatology-specific milestones. Using adult rheumatology's Milestones 2.0 as a starting point, the workgroup revised the patient care and medical knowledge subcompetencies and milestones to reflect requirements and nuances of pediatric rheumatology care. Milestones within four remaining competency domains (professionalism, interpersonal and communication skills, practice-based learning and improvement, and systems-based practice) were standardized across all pediatric subspecialties, and therefore not revised. The workgroup created a supplemental guide with explanations of the intent of each subcompetency, 25 in total, and examples for each milestone level. The new milestones are an important step forward for competency-based medical education in pediatric rheumatology. However, challenges remain. Milestone level assignment is meant to be informed by results of multiple assessment methods. The lack of pediatric rheumatology-specific assessment tools typically result in clinical competency committees determining trainee milestone levels without such collated results as the foundation of their assessments. Although further advances in pediatric rheumatology fellowship competency-based medical education are needed, Milestones 2.0 importantly establishes the first pediatric-specific rheumatology Milestones to assess fellow performance during training and help measure readiness for independent practice.

Case Report: Refractory macrophage activation syndrome requiring high-dose anakinra, emapalumab, and etoposide therapy in early-onset systemic juvenile idiopathic arthritis associated with adenoviremia.

Macrophage activation syndrome (MAS) is a life-threatening condition characterized by the excessive stimulation of macrophages and T lymphocytes, provoked by infections, malignancy, and autoimmune or autoinflammatory conditions such as systemic juvenile idiopathic arthritis (sJIA). Clinical signs of sJIA may include high-spiking, quotidian fevers, lymphadenopathy, hepatosplenomegaly, and a salmon-colored migratory, evanescent rash. By contrast, MAS is characterized by unremitting fevers and diffuse, fixed, maculopapular rashes. In addition to hepatosplenomegaly and lymphadenopathy, patients with MAS may also have clinical signs of coagulopathy, as well as cardiac, lung, renal, and central nervous system dysfunction. The empiric treatment for MAS is initially high-dose IV corticosteroids, but usually requires addition of immunomodulators such as tacrolimus or a biologic such as Anakinra to control. The addition of immunotherapies for MAS has improved patient outcomes. We present a 2-year-old male patient with a history of early-onset sJIA, who presented with MAS refractory to corticosteroids and anakinra triggered by adenoviremia that required addition of emapalumab to control. We believe this is the first reported case of a combination of immunosuppressive therapy of emapalumab, etoposide, anakinra, tacrolimus, and corticosteroids used in the successful treatment of infection-induced MAS in early-onset sJIA. Given the lack of treatment guidelines and approved therapies for MAS, alternative strategies should be considered for patients with an intractable course.

The relationships of kinesiophobia and physical function and physical activity level in juvenile idiopathic arthritis.

BACKGROUND: Kinesiophobia may hinder physical performance measures and functional quality of life in children with juvenile idiopathic arthritis (JIA). This study aims to quantify differences in physical function in patients with JIA compared to healthy controls, and determine the effects of kinesiophobia on physical function and physical activity. METHODS: This was a comparative study of participants with JIA and healthy controls (JIA n = 26, control n = 17). All children with JIA had lower extremity joint involvement. Performance-based measures included gait speed, chair and stair navigation performance. Self-reported measures included Patient Reported Outcome Measurement Information System (PROMIS®) Physical Function Mobility, and Pain Interference and the Pediatric Functional Activity Brief Scale (Pedi-FABS). The Tampa Scale of Kinesiophobia (TSK-11) assessed patient fear of movement due to pain. Linear regression models were used to determine the contribution of TSK-11 scores on performance test and Pedi-FABS scores. RESULTS: Gait speeds were 11-15% slower, chair rise repetitions were 28% fewer, and stair ascent and descent times were 26-31% slower in JIA than controls (p < .05). PROMIS® Physical Function Mobility scores were 10% lower and Pain Interference scores were 2.6 times higher in JIA than healthy controls (p = .003). TSK-11 scores were higher in JIA than controls (p < .0001). After controlling for covariates, TSK-11 scores explained 11.7-26.5% of the variance of regression models for stair climb time, chair rise performance and Pedi-FABS scores (p < .05). CONCLUSIONS: Children with JIA experience difficulty with tasks related to body transfers. Kinesiophobia is a significant contributor to the functional task performance and may impact clinical outcomes.

Gait parameters, functional performance and physical activity in active and inactive Juvenile Idiopathic Arthritis.

BACKGROUND: Children with Juvenile Idiopathic Arthritis (JIA) may adopt different movement patterns and participate in physical activity during different states of disease. RESEARCH QUESTION: Which specific features of gait and physical function performance differ among children with active or inactive JIA compared to healthy children? METHODS: Forty-three children participated (14.5 ± 4.2 yrs; 60 % female). 3D-motion analysis methods were coupled with force measures from an instrumented treadmill captured gait mechanical measures. The 30-second Chair Rise Test (repetitions) and stair ascent-descent tests were performed, and the 11-point Wong-Baker face scale assessed pain after each test. RESULTS: Compared to healthy controls children with active and inactive JIA had worse outcomes (12-21 % slower self-selected and fast walking speeds, 28-34 % slower stair navigation times, 28 % fewer chair rise repetitions in 30 s; all p < .05). Children with active JIA had 8-13 % slower gait speeds, 4 % fewer chair rise repetitions and 14-16 % slower stair navigation times. At faster walking speed, children with active JIA had less hip joint flexion/extension excursion in the sagittal plane during the gait cycle, produced higher leg stiffness, and demonstrated greater interlimb asymmetry in GRF vertical impulse during loading than healthy children (all p < .05). The Pedi-FABS subscore of "Duration: performing athletic activity for as long as you would like without stopping" was rated lower in children with active JIA compared to controls (p < .05). CONCLUSION: Gait speed, specific load-bearing functional tasks and leg stiffness features of gait may be informative 'functional biomarkers' for assessing JIA burden and tracking treatment efficacy. Additional prospective studies are needed to determine how these features change over time with pain change, and understand impact on quality of life and physical activity participation.

Juvenile idiopathic arthritis, gait characteristics and relation to function.

BACKGROUND: Juvenile Idiopathic Arthritis (JIA) is a chronic inflammatory arthritis that impacts biomechanical features of gait. This systematic review describes the effects of JIA on gait motion parameters and walking performance. METHODS: Six databases were searched (PubMed/Medline, Cochrane, the EBSCOHost database SPORTDiscus, Web of Science, and Embase). Studies were restricted to children with any subtype of JIA who were assessed for gait motion features (kinematic, kinetic, temporalspatial) or walking performance (velocity or distance covered); could include intervention or treatment exposure with measures of gait and gait speed; could involve comparison of gait in JIA to healthy controls. Quality of evidence was assessed using the GRADE system. This systematic review was registered at PROSPERO (CRD42018109582) RESULTS: The search yielded 625 papers, 23 of which described biomechanical features of gait and/or assessed walking performance. Twenty studies measured walking velocity and walking ability using simple field tests or laboratory methods. Eleven studies measured temporalspatial parameters such as cadence, step length, stride length, step width, single and double support time. Nine studies evaluated kinetic measurements including joint power, flexion and extension and joint moments. Nine studies evaluated kinematic parameters including range of motion, pelvic tilt, center of motion and trunk sway. CONCLUSIONS: Key features of gait in children with JIA include slower gait velocity, shortened step length, decreased range of motion at the hip, knee and ankle with trend towards flexion, decreased joint power, anteriorly tilted pelvis and trunk with shifted center of motion. There is a potential to ameliorate JIA-related gait changes with exercise and/or pharmaceutical interventions.