Treatment escalation patterns to start biologics in refractory moderate juvenile dermatomyositis among members of the Childhood Arthritis and Rheumatology Research Alliance.

Background: Despite new and better treatments for juvenile dermatomyositis (JDM), not all patients with moderate severity disease respond adequately to first-line therapy. Those with refractory disease remain at higher risk for disease and glucocorticoid-related complications. Biologic disease-modifying antirheumatic drugs (DMARDs) have become part of the arsenal of treatments for JDM.

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.

Objective: To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided.

Methods: We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions.

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Therapies, Medication Monitoring, Immunizations, and Imaging.

Objective: To provide recommendations for the management of juvenile idiopathic arthritis (JIA) with a focus on nonpharmacologic therapies, medication monitoring, immunizations, and imaging, irrespective of JIA phenotype.

Methods: We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low).

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.

Objective: To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided.

Methods: We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions.

Multi-pronged approach to enhance education of children and adolescents with lupus, caregivers, and healthcare providers in New Jersey: Needs assessment, evaluation, and development of educational materials.

Background: Childhood Systemic Lupus Erythematosus (cSLE) patients are younger at diagnosis and have a more severe disease course compared to adult onset SLE patients and develop significant complications related to disease and or immunosuppression. Moreover, female and minority populations experience higher rates of cSLE, with African American, Afro-Caribbean, and Hispanic populations being at greatest risk and having poor prognosis.

Methods: The Pediatric Alliance for Lupus initiative addressed the dearth in education and resources in a multi-stage process.

2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis.

Objective: To develop recommendations for the screening, monitoring, and treatment of uveitis in children with juvenile idiopathic arthritis (JIA).

Methods: Pediatric rheumatologists, ophthalmologists with expertise in uveitis, patient representatives, and methodologists generated key clinical questions to be addressed by this guideline. This was followed by a systematic literature review and rating of the available evidence according to the GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology.

2019 American College of Rheumatology/Arthritis Foundation Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Non-Systemic Polyarthritis, Sacroiliitis, and Enthesitis.

Objective: To develop treatment recommendations for children with juvenile idiopathic arthritis manifesting as non-systemic polyarthritis, sacroiliitis, or enthesitis.

Methods: The Patient/Population, Intervention, Comparison, and Outcomes (PICO) questions were developed and refined by members of the guideline development teams. A systematic review was conducted to compile evidence for the benefits and harms associated with treatments for these conditions.

2019 American College of Rheumatology/Arthritis Foundation Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Non-Systemic Polyarthritis, Sacroiliitis, and Enthesitis.

Objective: To develop treatment recommendations for children with juvenile idiopathic arthritis manifesting as non-systemic polyarthritis, sacroiliitis, or enthesitis.

Methods: The Patient/Population, Intervention, Comparison, and Outcomes (PICO) questions were developed and refined by members of the guideline development teams. A systematic review was conducted to compile evidence for the benefits and harms associated with treatments for these conditions.

2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis.

Objective: To develop recommendations for the screening, monitoring, and treatment of uveitis in children with juvenile idiopathic arthritis (JIA).

Methods: Pediatric rheumatologists, ophthalmologists with expertise in uveitis, patient representatives, and methodologists generated key clinical questions to be addressed by this guideline. This was followed by a systematic literature review and rating of the available evidence according to the GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology.

Biologic therapies for refractory juvenile dermatomyositis: five years of experience of the Childhood Arthritis and Rheumatology Research Alliance in North America.

Background: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported.

Novel method to collect medication adverse events in juvenile arthritis: results from the childhood arthritis and rheumatology research alliance enhanced drug safety surveillance project.

Objective: Few data are available regarding the rates of serious adverse events (SAEs) and important medical events (IMEs) outside of product-based registries and clinical trials for juvenile idiopathic arthritis (JIA). The Enhanced Drug Safety Surveillance Project (EDSSP) was developed to pilot a novel system to collect SAEs/IMEs in children with JIA. This analysis reports the results from this 4-year (2008-2012) EDSSP.

Consensus treatments for moderate juvenile dermatomyositis: beyond the first two months. Results of the second Childhood Arthritis and Rheumatology Research Alliance consensus conference.

Objective: To use consensus methods and the considerable expertise contained within the Childhood Arthritis and Rheumatology Research Alliance (CARRA) organization to extend the 3 previously developed treatment plans for moderate juvenile dermatomyositis (DM) to span the full course of treatment.

Methods: A consensus meeting was held in Chicago on April 23-24, 2010, involving 30 pediatric rheumatologists and 4 lay participants. Nominal group technique was used to achieve consensus on treatment plans that represented typical management of moderate juvenile DM.

Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children.

Objective: To evaluate demographic and clinical characteristics, duration of time between disease onset (date of first rash and/or weakness), and diagnosis/therapy, as well as socioeconomic status, of children with newly diagnosed juvenile dermatomyositis (JDM).

Methods: Structured telephone interview of families of a cohort of 79 children with JDM: interval between onset of symptoms to diagnosis, median of 3 months (range 0.5-20.

New-onset juvenile dermatomyositis: comparisons with a healthy cohort and children with juvenile rheumatoid arthritis.

Objective: To determine, in a case-control study, if patients with new-onset juvenile dermatomyositis (juvenile DM) have increased symptoms prior to onset, exposure to certain environmental conditions, frequency of familial autoimmune diseases, or antibody titers, compared with 2 control groups.

Methods: A structured interview with the families of 80 children with juvenile DM, 40 children with juvenile rheumatoid arthritis (JRA), or 23 healthy children, from the same geographic area as the children with juvenile DM, was conducted. All children's sera were tested for antibody to Toxoplasma gondii, herpes simplex virus (HSV), or coxsackievirus B (CVB).

Psychosocial effects of juvenile rheumatic disease. The family and peer systems as a context for coping.

The psychosocial effects of juvenile rheumatic diseases and disease activity were examined among 24 children and their families (12 children with a rheumatic disease and 12 children with no chronic illness). Each child with rheumatic illness was paired with a healthy control child nominated by their classroom teacher. Family and child functioning was assessed through measures of competence, coping, and adjustment and through direct observation of social functioning with peers at school.

Dark skin discoloration of finger joints in juvenile arthritis.

We examined the frequency of a known but poorly described finding, darkened skin over the proximal interphalangeal joints in patients with polyarticular juvenile arthritis. Examining a consecutive group of patients we found that 77% showed this sign. It reflects the presence of or previous episodes of inflammation of these joints.

Physical maturity screening for participation in sports.

In an effort to develop screening criteria to allow nonphysicians to detect physically immature boys prior to interscholastic sports competition, 364 male adolescents were studied as part of their group preparticipation health evaluation. Handgrip and self-assessed Tanner stage were measured, in addition to the routine preparticipation health evaluation procedures. Of the 118 boys who were Tanner 3 or less (immature), 103 (87%) had weak grips (less than 55 lb [24.

Thiemann's disease.

Two cases of Thiemann's disease in children are reported for the first time from North America. A history of swollen, tender proximal interphalangeal joints with radiographic evidence of irregularities of the epiphyses leading to premature fusion and subsequent shortening of the middle phalanges resulted in this diagnosis. Recognition of these features should lead to its more frequent diagnosis.

The hypermobility syndrome.

The hypermobility syndrome has been recognized as a definitive diagnostic entity among children referred to a Pediatric Arthritis Clinic with musculoskeletal complaints. The diagnosis of hypermobility was made by the ability of the patients to perform at least three of the following maneuvers: (1) extension of the wrists and metacarpal phalanges so that the fingers are parallel to the dorsum of the forearm; (2) passive apposition of thumbs to the flexor aspect of the forearm; (3) hyperextension of elbows (greater than or equal to 10 degrees); (4) hyperextension of knees (greater than or equal to 10 degrees); (5) flexion of trunk with knees extended so palms rest on the floor. Of 262 patients, 15 (5.

The prevalence of juvenile arthritis.

A secondary analysis of two new large practitioner surveys, one national and one local, showed the prevalence of juvenile arthritis to be between 0.16 and 0.43 cases per 1,000 children.