Patients With IgA Vasculitis and Kawasaki Disease Show Dysregulated Interferon Signature

IgA vasculitis (IgAV) and Kawasaki disease (KD) are the most common forms of childhood vasculitis. Although various factors such as viral infections, genetic factors, and environmental factors are involved in the development of both diseases, their pathogenesis remains unclear. The interferon (IFN) signature, which reflects the activation of type I IFN signaling pathways, is a diagnostic and prognostic tool that contributes significantly to the pathogenesis and management of autoimmune diseases. In our study, we aimed to investigate the role of the IFN signature in patients with IgAV and KD.

Thirty-two children with IgAV and four patients diagnosed with KD were included in the study. Serum levels of IL-1, IL-6, IL-8, IL-10, IL-17, IL-18, TNF-α, TNF-R1, TNF-R2, and IFN-gamma were analyzed in the serum samples of all participants, and the expression of IFN-related genes (STAT1, IFI27, IFI44, IFI44L, IFIT1, and RSAD2) was assessed in the patients and healthy controls (n = 26) to calculate the IFN score by RT-PCR method.

A significant increase in the expression of three genes (IFIT1, IFI44, and IFI27) and decreased expression of the other three genes (RSAD2, STAT1, and IFI44L) was found in patients with IgAV and KD compared to the control group. Significantly higher IFN scores (IFN > 3) were found in patients with gastrointestinal involvement, in patients who required corticosteroid therapy, and in patients who had to be hospitalized. No significant difference in IFN levels was found between patients with and without renal involvement. Significantly higher serum IL-1 levels were found in patients with gastrointestinal symptoms with IgAV. High IFN scores were found in three out of four patients diagnosed with KD.

A dysregulated type 1 IFN signature was found in patients with IgAV and KD compared to the control group. A significantly increased risk of gastrointestinal involvement required corticosteroid therapy, and hospitalization was observed in patients diagnosed with IgAV who had high IFN levels. This underlines the idea that the IFN score could serve as a crucial prognostic indicator.