Peripheral blood cytokine and chemokine profiles in juvenile localized scleroderma: T-helper cell-associated cytokine profiles

Objective: To evaluate peripheral blood T-helper (T-H) cell-associated cytokine and chemokine profiles in localized scleroderma (LS). and correlate them with clinical disease features, including disease activity parameters. Methods: A 29-plex Luminex platform was used to analyze the humoral profile of plasma samples from 69 pediatric LS patients and 71 healthy pediatric controls. Cytokine/chemokine levels were compared between these two groups and within LS patients, focusing on validated clinical outcome measures of disease activity and damage in LS. Results: Plasma levels of IP-10, MCP-1, IL-17a, IL-12p70, GM-CSF, PDGF-bb, IFN-alpha 2, and IFN-gamma were significantly higher in LS subjects compared to healthy controls. Analysis within the LS group demonstrated IP-10, TNF-alpha, and GM-CSF correlated with clinical measures of disease activity. Several cytokines/chemokines correlated with anti-histone antibody, while only a few correlated with positive ANA and single-stranded DNA antibody. Conclusion: This is the first time that multiple cytokines and chemokines have been examined simultaneously in LS. In general, a T(H)1 (IFN-gamma) and T(H)17 (IL-17a) predominance was demonstrated in LS compared to healthy controls. There is also an IFN-gamma signature with elevated IP-10, MCP-1, and IFN-gamma, which has been previously demonstrated in systemic sclerosis, suggesting a shared pathophysiology. Within the LS patients, those with active disease demonstrated IP-10, TNF-alpha, and GM-CSF, which may potentially serve as biomarkers of disease activity in the clinical setting. (C) 2015 Elsevier Inc. All rights reserved.