Research

The Intermountain Database for Childhood Rheumatic Diseases.  Dr. Bohnsack is the primary investigator for the database, which is a collection of biologic samples  and clinical data maintained for the purpose of identifying the genetic and environmental causes of juvenile arthritis and other childhood rheumatic diseases.

Samples from the Intermountain Database are being used in NIH-funded Genome-Wide Association Studies (GWAS) at Cincinnati Children’s Hospital to identify genetic variants that predispose to disease in children with rheumatoid factor-negative polyarticular  and oligoarticular JIA. Samples have also been provided to the NIH for a GWAS of Systemic Onset JIA (the IN CHARGE study). 

Dr. Bohnsack is also co-investigator with Dr. Sampath Prahalad  (Principal Investigator, Emory University) on an NIH-funded RO-1 entitled Segmental Chromosome Sharing in Affected Relatives with JIA.  The aim of this study is to identify regions of the genome with excessive chromosomal segmental sharing in affected individuals in extended multiplex JIA pedigrees, and to identify variants involved in JIA susceptibility. These pedigrees were identified in Utah using the resources of the Utah Population Database.

Pediatric Lupus Outcomes Study (PLOS). Dr. Aimee Hersh is the primary investigator for the Pediatric Lupus Outcomes Study (PLOS), a longitudinal survey study designed to assess the long-term medical, functional outcomes of adults diagnosed with Systemic Lupus Erythematosus in childhood. Dr. Hersh is also investigating the impact of the transition from pediatric to adult rheumatology care and change in health insurance coverage on the long-term health outcomes of pediatric Lupus patients.

Clinical Research Informatics. Dr. CJ Inman is investigating the use of Registries for Clinical Research. Dr. Inman’s focus is on the data quality of Clinical Registries and applying informatics based techniques to improve the quality of data collected in these Registries.

Dr.Rafael Firszt is the lead investigator on a project researching Food Protein-Induced Enterocolitis Syndrome following Enteral Exposure to Shrimp Protein in Humans. Dr. Rafael Firszt will also be investigating the Underlying Mechanisms of Chronic Idiopathic Urticaria in children and adults with the goal of better understanding the disease and identifying biomarkers to help guide management.

Genomics of Multiple Intestinal Atresia with Immune Deficiency,  and other inherited Immune Deficiency Disorders.  Dr. Karin Chen is using whole genome and whole exome sequencing to identify mutations in genes that cause or modify inherited immune deficiency diseases.

Current Clinical Studies 

Follow Up of Subjects from the TRIAL of EARLY AGGRESSIVE THERAPY in JUVENILE IDIOPATHIC ARTHRITIS (TREAT Follow Up)  This is a multi-center, NIH-funded trial to determine the proportion of subjects that attain clinical remission during the observation time period, and the length of their disease states, compared with their initial treatment arm of the TREAT study.

A Multicenter, Double Blind, Randomized-Withdrawal Trial of Subcutaneous Golimumab, a Human Anti-TNFα Antibody, in Pediatric Subjects with Active Polyarticular Course Juvenile Idiopathic Arthritis (JIA) Despite Methotrexate Therapy (GoKids). This is a multicenter, industry-sponsored trial of the efficacy of Golimumab in patients with JIA.

A long-term, multi-center, longitudinal post-marketing observational Registry to Assess Long Term Safety and Effectiveness of Humira® (adalimumab) in children with Moderate to Severe Active Polyarticular or Polyarticular Course Juvenile Idiopathic Arthritis (JIA) – STRIVE. This is a 15 year, industry-sponsored prospective trial. The objective of this registry study is to evaluate the long-term safety and effectiveness of Humira® using Methotrexate (MTX) as a reference group in patients aged four to 17 years old with JIA.

Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. CARRAnet is an ARRA funded multicenter and multiple disease national registry in pediatric rheumatology. CARRAnet is associated with the Childhood Arthritis and Rheumatology Research Alliance which is the largest Pediatric Rheumatology research organization in North America.  Sixty sites will potentially participate allowing every CARRA center the opportunity to be involved in research. This is a unique opportunity to collect information on rare diseases from most pediatric rheumatology centers in the US. The information collected in the CARRAnet registry will allow creation of treatment protocols and research for a paradigm shift toward evidence based care in pediatric rheumatology.

ARCHIVE (A Registry for Children with Vasculitis: e-entry). This is a multi-center registry that collects clinical and demographic data in order to characterize the spectrum of disease manifestations in childhood systemic vasculitis.

Development of Autoimmunity in Puberty in Childhood SLE. This is a multicenter, prospective study designed to characterize the relationship of hormonal changes during puberty to the development of disease manifestations in children who develop SLE before puberty.

CARRA Enhanced Drug Safety Surveillance Project in Juvenile Rheumatoid and Idiopathic Arthritis (EDSS).  The goal of this multicenter study is to report possible Serious Adverse Events or Important Medical Events related to medication use in JIA patients and to ascertain the number of unique patients with JIA treated at each site.