Peter R. Blier

Consensus treatment plans for induction therapy of newly diagnosed proliferative lupus nephritis in juvenile systemic lupus erythematosus

To formulate consensus treatment plans (CTPs) for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in juvenile systemic lupus erythematosus (SLE).

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Systemic juvenile idiopathic arthritis (JIA) is characterized by fevers, rash, and arthritis, for which interleukin‐1 (IL‐1) and IL‐6 inhibitors appear to be effective treatments. Pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and alveolar proteinosis (AP) have recently been reported with increased frequency in systemic JIA patients. Our aim was to characterize and compare systemic JIA patients with these complications to a larger cohort of systemic JIA patients.

Race, Income, and Disease Outcomes in Juvenile Dermatomyositis

Objective To determine the relationships among race, income, and disease outcomes in children with juvenile dermatomyositis (JDM). Study design Data from 438 subjects with JDM enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were analyzed. Demographic data included age, sex, race, annual family income, and insurance status. Clinical outcomes included muscle strength, presence of rash, calcinosis, weakness, physical function, and quality of life measures. Disease outcomes were compared based on race and income. Results Minority subjects were significantly more likely to have low annual family income and significantly worse scores on measures of physical function, disease activity, and quality of life measures. Subjects with lower annual family income had worse scores on measures of physical function, disease activity, and quality of life scores, as well as weakness. Black subjects were more likely to have calcinosis. Despite these differences in outcome measures, there were no significant differences among the racial groups in time to diagnosis or duration of disease. Using calcinosis as a marker of disease morbidity, black race, annual family income <

Novel Method to Collect Medication Adverse Events in Juvenile Arthritis: Results From the Childhood Arthritis and Rheumatology Research Alliance Enhanced Drug Safety Surveillance Project

50 000 per year, negative antinuclear antibody, and delay in diagnosis >12 months were associated with calcinosis. Conclusion Minority race and lower family income are associated with worse morbidity and outcomes in subjects with JDM. Calcinosis was more common in black subjects. Further studies are needed to examine these associations in more detail, to support efforts to address health disparities in subjects with JDM and improve disease outcomes.

Juvenile Fibromyalgia: A Primary Pain, or Pain Processing, Disorder

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.

Novel Method to Collect Medication Adverse Events in Juvenile Arthritis: Results From the Childhood Arthritis and Rheumatology Research Alliance Enhanced Drug Safety Surveillance Project: Enhanced Drug Safety Surveillance Project

Juvenile fibromyalgia (JFM), a chronic disorder of widespread musculoskeletal pain in combination with autonomic, sensory, and cognitive dysfunction, is responsible for considerable morbidity and impaired quality of life in affected patients and their families. Historically, fibromyalgia has been incorrectly characterized as a psychosomatic or psychogenic disorder, but new understanding of the science of pain has demonstrated unambiguously that it is an organic disorder of the pain processing system itself. This new science provides a framework for understanding the pathophysiology of fibromyalgia and for developing rational therapeutic interventions. Advances in JFM include the verification of adult criteria for diagnosis in pediatric patients and the publication of effective therapies based on cognitive and physical neuromuscular intervention. Although primarily nonpharmacologic therapy can include adjunctive medications as well. Finally, the recognition that JFM is a disorder of the central and peripheral nervous systems suggests that neurologists can be important in the care of these patients.

Novel method to collect medication adverse events in juvenile arthritis

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.

SOLUBLE ADHESION MOLECULES IN JUVENILE RHEUMA TOID ARTHRITIS • 1838

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.

Soluble adhesion molecules in juvenile rheumatoid arthritis.

Cell surface adhesion molecules mediate important cellular interactions in acute and chronic inflammatory responses. Soluble forms of a number of adhesion molecules are generated either by proteolytic cleavage or alternate mRNA splicing. The function of soluble adhesion molecules is unknown, but they may play a regulatory role in inflammatory processes. Studies of these molecules in adult autoimmune diseases have furthered our understanding of these conditions, but such studies have been lacking in juvenile rheumatoid arthritis (JRA). We therefore studied serum levels of soluble forms (s) of ICAM-1, E-selectin, L-selectin, VCAM-1, and ICAM-3 by sandwich ELISA in 16 children with JRA (6 systemic, 6 polyarticular, 4 pauciarticular). We analyzed differences in levels of these molecules among JRA subtypes by ANOVA, and also correlated these levels with erythrocyte sedimentation rate (ESR), hematocrit, white blood cell count (WBC), and total platelet (PLT) counts, joint count, and duration of morning stiffness by linear regression analysis.RESULTS: sE-selectin levels were significantly higher in patients with systemic disease as compared to other subtypes (p<.04). Furthermore, there was a trend toward higher levels of sICAM-1 in systemic disease, but the difference did not reach statistical significance. Significant correlations were found between sE-selectin and ESR(r=.68,p<.006), WBC (r=.70,p<.003), and PLT (r=.54,p<.05), and between sL-selectin and WBC (r=.55,p<.03). We conclude that levels of sE-selectin, and possibly sICAM-1 are relatively elevated in systemic JRA, and may indicate cytokine induction and endothelial cell activation in that subtype. Several molecules, especially sE-selectin, correlated with hematologic parameters inJRA. This suggests that serum levels of these molecules may provide a useful additional marker for disease activity in certain patients.Funded by Boehringer Ingelheim Pharmaceuticals