ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2472

Highly Elevated S100A8/A9 and S100A12 Levels May Distinguish Systemic Juvenile Idiopathic Arthritis Patients with New Onset Disease and Subclinical Macrophage Activation Syndrome

Jessica Turnier1, Ndate Fall2, Alexei A. Grom3, Sherry Thornton2 and Hermine I. Brunner4, 1Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Division of Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, 3PRCSG, Cincinnati, OH, 4Cincinnati Children's Hospital Medical Center, Cincinnati, OH

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords:

Session Information

Date: Tuesday, November 10, 2015

Title: Pediatric Rheumatology - Pathogenesis and Genetics Poster

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Systemic juvenile idiopathic arthritis remains a clinical diagnosis without a specific diagnostic test.  Previous studies have demonstrated that extremely high levels of S100 proteins may distinguish patients with systemic juvenile idiopathic arthritis (sJIA) from patients with other inflammatory diseases or additional subtypes of juvenile idiopathic arthritis.  A great degree of heterogeneity exists within the serum levels of S100 proteins amongst sJIA patients.  The purpose of this study was to explore the degree of variability within S100 protein levels in sJIA patients and to correlate S100 protein levels with clinical presentation. 

Methods:

A total of 189 serum samples were collected from patients with different subtypes of juvenile idiopathic arthritis, including sJIA (n=22), persistent oligoarthritis (n=28), extended oligoarthritis (n=28), rheumatoid factor positive polyarthritis (n=27), rheumatoid factor negative polyarthritis (n=28), enthesitis-related arthritis (n=29) and psoriatic arthritis (n=27).  In addition, patients with sJIA were further divided into active sJIA (n=11) and clinically inactive disease (n=11).  Control samples (n=90) were obtained from healthy children aged 2-18 years old.  Serum samples were analyzed for protein levels of S100A8/A9 and S100A12 using commercial ELISAs (BÜHLMANN MRP8/14 and CircuLex S100A12/EN-RAGE).  Clinical characteristics of the sJIA patients were examined for differences in symptoms, exam findings, lab values and treatment at the time of sample collection. 

Results:

Patients with active sJIA had the highest levels of S100 proteins when compared to other subtypes of juvenile idiopathic arthritis.  Patients with clinically inactive sJIA had S100 protein levels comparable to controls.  A bimodal distribution was noted in both the S100A8/A9 and S100A12 levels in patients with active sJIA.  Systemic juvenile idiopathic arthritis patients in the subgroup with higher levels of S100 proteins were determined to have new onset disease as well as subclinical features of macrophage activation syndrome (low hemoglobin, elevated ferritin, hepatosplenomegaly, relatively low platelets in the setting of elevated CRP).  The fold difference between protein levels in new onset sJIA versus active sJIA was 29 for the S100A8/A9 levels and 31 for the S100A12 levels.

Conclusion:

Higher levels of S100A8/A9 and S100A12 proteins correlate with disease activity in systemic juvenile idiopathic arthritis as previously described.  Marked heterogeneity exists in S100 protein levels amongst active sJIA patients.  Active sJIA patients in the subgroup with significantly higher levels of S100 proteins could represent a unique disease subtype.

Disclosure: J. Turnier, None; N. Fall, None; A. A. Grom, Novartis, Roche, 5; S. Thornton, None; H. I. Brunner, None.

To cite this abstract in AMA style:

Turnier J, Fall N, Grom AA, Thornton S, Brunner HI. Highly Elevated S100A8/A9 and S100A12 Levels May Distinguish Systemic Juvenile Idiopathic Arthritis Patients with New Onset Disease and Subclinical Macrophage Activation Syndrome [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/highly-elevated-s100a8a9-and-s100a12-levels-may-distinguish-systemic-juvenile-idiopathic-arthritis-patients-with-new-onset-disease-and-subclinical-macrophage-activation-syndrome/. Accessed .

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/highly-elevated-s100a8a9-and-s100a12-levels-may-distinguish-systemic-juvenile-idiopathic-arthritis-patients-with-new-onset-disease-and-subclinical-macrophage-activation-syndrome/