Needs Assessment of Musculoskeletal Ultrasound Training in North American Pediatric Rheumatology Fellowship Programs.

OBJECTIVE: Musculoskeletal ultrasound (MSUS) is widely used in adult rheumatology practice for diagnosis of arthritis and procedural guidance; however, it is not yet common practice in pediatric rheumatology. MSUS is advantageous to the pediatric population because it lacks radiation and eliminates need for sedation. This study aims to assess interest in, access to, and barriers to MSUS training in pediatric rheumatology fellowship programs in North America. METHODS: A survey was developed by pediatric rheumatology providers with experience in medical and/or MSUS education and distributed via REDCap anonymously in March 2022 (Supplementary Material). Eligible participants included current and recently graduated (<1 year) pediatric rheumatology fellows at a North American program. Descriptive statistics and bivariate analyses using design-based Pearson chi-squared tests were performed. RESULTS: Overall response rate was 78% (88/113), and 75% reported some form of MSUS training during fellowship. Only 36% indicated their program had a formal MSUS curriculum. Of those with MSUS training, 23% reported adult-only MSUS education. Eighty-four percent felt MSUS would be beneficial to their career. Major barriers to MSUS training included lack of MSUS-trained faculty, lack of time, and lack of hands-on MSUS sessions. Those who had access to MSUS training were significantly more interested in MSUS than those without (P = 0.0036). CONCLUSION: This study demonstrates that North American pediatric rheumatology fellows have a strong interest in learning MSUS, but they face significant challenges in accessing MSUS training (lack of MSUS-trained faculty, time, and access to hands-on training). MSUS should be incorporated into fellowship curriculum; however, implementation remains a challenge.

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.

Comparison of efficacy between triamcinolone acetonide and triamcinolone hexacetonide for intraarticular therapy in juvenile idiopathic arthritis: a retrospective analysis.

BACKGROUND: There are many FDA-approved corticosteroid preparations available for intra-articular injection, however triamcinolone hexacetonide is not one of them. It was the intraarticular drug of choice among pediatric rheumatologists up until approximately a decade ago, when production of this medication ceased. It can be obtained in the United States and Canada via importation from Europe, but it is not FDA-approved at this time. We wish to compare the duration of remission of intraarticular triamcinolone hexacetonide (TH) with that of triamcinolone acetonide (TA) in children with juvenile idiopathic arthritis (JIA) and demonstrate its safety in this population. METHODS: This retrospective chart review included 39 patients with JIA who received intraarticular corticosteroid injections (IACIs) from September 2018 to September 2019. These patients were reviewed and their life-time injections with either TH (41 joints) or TA (124 joints) was noted through May 30, 2021. Patients with concomitant systemic therapy initiation were excluded. The primary outcome was time to relapse. Relapse was defined by the presence of arthritis on physical examination by an attending rheumatologist. Kaplan-Meier curves and a log-rank test were constructed to compare the probability of time to relapse between IACI injections. Additionally, mixed effects cox regression models were constructed to account for multiple injections per participant. RESULTS: Kaplan-Meier estimator of median relapse time in months was higher for TH. Based on the log-rank test, TA joints had a higher probability of experiencing a relapse during the study time (p value < 0.001). The hazard of time to relapse was reduced when comparing TH to TA in both unadjusted and adjusted mixed effects cox regression models [unadjusted hazard ratio (95% confidence interval): 0.184 (0.089, 0.381); adjusted hazard ratio (95% confidence interval): 0.189 (0.092, 0.386)]. CONCLUSIONS: TH has longer duration of action than TA and is associated with less systemic side effects. It should be considered the drug of choice for intraarticular corticosteroid injections in children with JIA.

Physical Therapy Interventions and Outcome Measures for a Patient Diagnosed with Anti-NMDA Receptor Encephalitis.

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is the most common cause of autoimmune encephalitis after acute demyelinating encephalitis. Patients usually present with acute behavioral changes, psychosis, and abnormal limb movements and can also present with symptoms of catatonia. Treatment typically consists of an immunotherapy protocol consisting of intravenous immunoglobulin, corticosteroids, and plasmapheresis. This article describes the medical treatment, physical therapy (PT) interventions, and outcomes of a 16-year-old patient with anti-NMDA receptor encephalitis with malignant catatonia. Using PT interventions such as bed mobility, transfer, and gait training, and parent education initially, and progressing to balance re-education and age-appropriate functional tasks the patient was able to progress and be discharged to home with family with a recommendation for continued PT treatment in the outpatient setting. [Pediatr Ann. 2021;50(10):e437-e443.].

Applications of Musculoskeletal Ultrasound in the Pediatric Emergency Department.

Musculoskeletal ultrasound is an emerging point-of-care ultrasound (POCUS) imaging modality that can be used to assess patients who present to the pediatric emergency department with musculoskeletal complaints. It is useful in detecting effusions resulting from infection or injury and can also help identify fractures and foreign bodies. Ultrasound is particularly useful in guiding joint aspiration and foreign body removal. This article reviews the role of POCUS in evaluating for hip, knee, ankle, and elbow effusions, long bone fractures, and foreign bodies. [Pediatr Ann. 2021;50(10):e411-e418.].

Anasarca as the presenting symptom of juvenile dermatomyositis: a case series.

BACKGROUND: Juvenile Dermatomyositis (JDM) is an autoimmune disease that typically presents with classic skin rashes and proximal muscle weakness. Anasarca is a rare manifestation of this disease and is associated with a more severe and refractory course, requiring increased immunosuppression. Early recognition of this atypical presentation of JDM may lead to earlier treatment and better outcomes. CASE PRESENTATION: We present two female patients, ages 11 years old and 4 years old, who presented to the ED with anasarca and were subsequently diagnosed with JDM. Both patients required ICU-level care and significant immunosuppression, including prolonged courses of IV methylprednisolone, IVIG, and Rituximab. CONCLUSIONS: Anasarca is a rare presentation of Juvenile Dermatomyositis, but it is important for clinicians to recognize this manifestation of the disease. Early recognition and treatment will lead to better outcomes in these children and hopefully decrease the need for prolonged hospitalization and ICU level care.

Juvenile Systemic Lupus Erythematosus Presenting with Esophagitis and Severe Oral Mucositis.

We present a case of a previously healthy adolescent female who developed severe oral mucositis and acute esophagitis as her presenting symptoms of juvenile systemic lupus erythematosus. Mucositis involving the lips is infrequently reported in systemic lupus erythematosus, and to our knowledge, this is the first reported case of acute, non-infectious esophagitis as a presenting symptom in a pediatric systemic lupus erythematosus patient.

Novel Ultrasound Image Acquisition Protocol and Scoring System for the Pediatric Knee.

OBJECTIVE: The use of musculoskeletal ultrasound is increasing among pediatric rheumatologists. Reliable scoring systems are needed for the objective assessment of synovitis. The aims of this study were to create a standardized and reproducible image acquisition protocol for B-mode and Doppler ultrasound of the pediatric knee, and to develop a standardized scoring system and determine its reliability for pediatric knee synovitis. METHODS: Six pediatric rheumatologists developed a set of standard views for knee assessment in children with juvenile arthritis. Subsequently, a comprehensive literature review, practical exercises, and a consensus process were performed. A scoring system for both B-mode and Doppler was then developed and assessed for reliability. Interreader reliability or agreement among a total of 16 raters was determined using 2-way single-score intraclass correlation coefficient (ICC) analysis. RESULTS: Twenty-one views to assess knee arthritis were initially identified. Following completion of practical exercises and subsequent consensus processes, 3 views in both B-mode and Doppler were selected: suprapatellar longitudinal and medial/lateral parapatellar transverse views. Several rounds of scoring and modifications resulted in a final ICC of suprapatellar view B-mode 0.89 (95% confidence interval [95% CI] 0.86-0.92) and Doppler 0.55 (95% CI 0.41-0.69), medial parapatellar view B-mode 0.76 (95% CI 0.68-0.83) and Doppler 0.75 (95% CI 0.66-0.83), and lateral parapatellar view B-mode 0.82 (95% CI 0.75-0.88) and Doppler 0.76 (95% CI 0.66-0.84). CONCLUSION: A novel B-mode and Doppler image acquisition and scoring system for assessing synovitis in the pediatric knee was successfully developed through practical exercises and a consensus process. Study results demonstrate overall good-to-excellent reliability.

Intravenous Immunoglobulin in the Treatment of Primary Immunodeficiency Diseases.

Intravenous immunoglobulin (IVIG) has been used as antibody replacement therapy in primary immunodeficiency diseases (PIDDs) for more than 50 years. Its role as a therapeutic agent has expanded over the past couple of decades as its anti-inflammatory and immune-modulatory mechanisms of action have been elucidated. It is now used "off-label" to treat other autoimmune diseases. This article focuses on the role of IVIG in the treatment of PIDDs characterized by absent or deficient antibody production. Replacement doses are given on a monthly basis in these conditions as a prophylactic measure to prevent acute and serious bacterial infections. [Pediatr Ann. 2017;46(1):e8-e12.].

Great Vessels of Children: Takayasu's Arteritis.

Takayasu's arteritis (TA) is a granulomatous, large vessel vasculitis affecting primarily the aorta and its main branches. It is characterized by inflammation in the blood vessel wall, leading to either luminal occlusion or dilatation with aneurysm formation. The etiology of TA is unknown, but there seems to be a strong role for cell-mediated autoimmunity in the pathogenesis of this disease. TA most commonly presents in young women in their second and third decades of life, but has been reported in children as young as age 2 years. The symptoms can range from vague systemic complaints to catastrophic stroke. Angiography remains the gold standard for diagnosis, although computed tomography angiography and magnetic resonance angiography have been used as well. Corticosteroids are first-line therapy, with various cytotoxic medications being used in refractory disease. Biologic agents targeting cytokines that are involved in disease pathogenesis have also been used successfully. In this article, we describe a patient with TA who responded to therapy with infliximab, an inhibitor of tumor necrosis factor.