Adam M. Huber

Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients

To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA).

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegeners) (GPA).

2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

To develop response criteria for juvenile dermatomyositis (DM).

Childhood Arthritis and Rheumatology Research Alliance Consensus Clinical Treatment Plans for Juvenile Dermatomyositis with Persistent Skin Rash

Objective. Juvenile dermatomyositis (JDM) is the most common form of idiopathic inflammatory myopathy in children. While outcomes are generally thought to be good, persistence of skin rash is a common problem. The goal of this study was to describe the development of clinical treatment plans (CTP) for children with JDM characterized by persistent skin rash despite complete resolution of muscle involvement. Methods. The Childhood Arthritis and Rheumatology Research Alliance, a North American consortium of pediatric rheumatologists and other healthcare providers, used a combination of Delphi surveys and nominal group consensus meetings to develop CTP that reflected consensus on typical treatments for patients with JDM with persistent skin rash. Results. Consensus was reached on patient characteristics and outcome assessment. Patients should have previously received corticosteroids and methotrexate (MTX). Three consensus treatment plans were developed. Plan A added intravenous immunoglobulin (IVIG) if it was not already being used. Plan B added mycophenolate mofetil, while Plan C added cyclosporine. Continuation of previous treatments, including corticosteroids, MTX, and IVIG, was permitted in plans B and C. Conclusion. Three consensus CTP were developed for use in children with JDM and persistent skin rash despite complete resolution of muscle disease. These CTP reflect typical treatment approaches and are not to be considered treatment recommendations or standard of care. Using prospective data collection and statistical methods to account for nonrandom treatment assignment, it is expected that these CTP will be used to allow treatment comparisons, and ultimately determine the best treatment for these patients.

Race is a risk factor for calcinosis in patients with JDM – early results from the CARRAnet registry study

Purpose Previously established risk factors for calcinosis include duration of disease and the length of time to treatment, suggesting early aggressive treatment may be important in preventing calcinosis. Race has also been suggested to be a risk factor, but this observation was thought to be secondary to delays in treatment. We utilized the CARRAnet registry to investigate potential risk factors for calcinosis.

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

To characterize the early disease course in childhood‐onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12‐month outcomes in children with AAV.

Idiopathic Inflammatory Myopathies in Childhood: Current Concepts

The juvenile idiopathic inflammatory myopathies are a group of rare, chronic, multisystem, autoimmune diseases associated with muscle weakness. This article reviews practical issues of relevance to pediatricians, including clinical presentation, differential diagnosis, investigation, therapy, and prognosis. The importance of early recognition and specialist involvement is emphasized.

Cognitive Performance Scores for the Pediatric Automated Neuropsychological Assessment Metrics in Childhood-Onset Systemic Lupus Erythematosus.

To develop and initially validate a global cognitive performance score (CPS) for the Pediatric Automated Neuropsychological Assessment Metrics (PedANAM) to serve as a screening tool of cognition in childhood lupus.

Update on the Assessment of Children with Juvenile Idiopathic Inflammatory Myopathy

The availability of validated measurement tools to assess children with juvenile idiopathic inflammatory myopathies is critical to clinical care and to ongoing research into new therapeutic modalities and approaches. Recent work has led to the development of tools for assessment of muscle strength and physical function, skin disease, global disease activity and damage, extramuscular disease, and quality of life. Ongoing work is attempting to integrate these assessments into core sets and definitions of improvement and flare. Future work will refine these assessments and definitions and—where there are multiple choices—determine which tools constitute an optimal assessment.

Health-Related Quality of Life in an Inception Cohort of Children With Juvenile Idiopathic Arthritis: A Longitudinal Analysis.

To describe changes in health‐related quality of life (HRQoL) over time in children with juvenile idiopathic arthritis (JIA), relative to other outcomes, and to identify predictors of unfavorable HRQoL trajectories.