Forty-one Cytokine Profile and Interferon-I Score in Juvenile Dermatomyositis: A Case Series Study

To analyze a broad spectrum of cytokine in the serum of patients with JDM.

Juvenile dermatomyositis (JDM) is the most common subtype of idiopathic inflammatory myopathies characterized by muscle and skin involvement. The etiology of JDM is unclear. A variety of cytokines play a role in the pathogenesis of JDM. Interferons, galectin-9, CLCX10, and neopterin are the most promising biomarkers.

This study describes the associations between clinical symptoms, cytokine, and interferon profiles in children with JDM.

Ten patients (6 girls and 4 boys) with JDM were included in the study. The clinical symptoms, disease activity (CMAS, CAT), laboratory parameters, and treatment were assessed. Forty-one cytokines levels and IFN-I scores in the serum were measured. The levels of cytokines were compared with a group of healthy controls (n=25).

Significant differences were observed in 21 of 41 analyzed cytokines between JDM patients and healthy controls. Patients with active disease (n=8) have higher levels of fractalkine (p = 0.036), IFNa (p = 0.037), IFNg (p = 0.037), GRO (p = 0.037), IL-10 (p = 0.037), IL-12p40 (p = 0.037), IL-12p70 (p = 0.048), IL-17a (p = 0.048), IL-1RA (p = 0.037), IL-1a (p = 0.037), compared to patients with inactive disease (n=2). A strong positive association was found between aCAT activity and eotaxin (r=0.753, p =0.012), GRO (r=0.735, p =0.015), IP-10 (r=0.805, p =0.005), and MCP-1 (r=0.734, p =0.016). A strong negative correlation association was observed between CMAS and eotaxin (r= -0.714, p =0.020), GRO (r= -0.727, p =0.017), IL-10 (r= -0.786, p =0.007), IP-10 (r= - 0.719, p =0.019), and MCP-1 (r= -0.800, p =0.005). IFN-I scores showed a positive correlation with IFNa (r=0.790, p =0.007), GRO (r=0.736, p =0.015) and IL-1RA (r=0.930, p <0.001).

Among the spectrum of 41 cytokines, GRO, eotaxin, IP-10, and MCP-1 have shown the strongest association with JDM activity.