Decreased levels of histidine-rich glycoprotein and increased levels of high-mobility group box 1 are risk factors for refractory Kawasaki disease

• Published in: Modern rheumatology 2023-04, Vol.33 (3), p.599-607
• Main Authors: Namba, Takahiro, Yashiro, Masato, Fujii, Yosuke, Tsuge, Mitsuru, Liu, Keyue, Nishibori, Masahiro, Tsukahara, Hirokazu
• Format: Article
• Language: English
• Subjects: HMGB1 Protein - therapeutic use; Humans; Mucocutaneous Lymph Node Syndrome - diagnosis; Mucocutaneous Lymph Node Syndrome - drug therapy; Prospective Studies; Risk Factors
• ISSN: 1439-7595; 1439-7609
• DOI: 10.1093/mr/roac040

ABSTRACT Objectives Histidine-rich glycoprotein (HRG) and high-mobility group box 1 (HMGB1) regulate the activation of neutrophils and vascular endothelium. The aim of this study was to quantify HRG and HMGB1 levels in patients with Kawasaki disease (KD) and evaluate their use in the clinical management of KD. Methods This study was prospectively performed. Patients were divided into two groups and analysed depending on whether KD symptoms improved by Day 10 of illness. HRG, HMGB1, and other laboratory variables were measured before the first treatment in all cases and, in most cases, afterwards for assessing trends. Results In this prospective study, we enrolled 60 patients with KD and 48 healthy controls. The HRG level in the KD group was significantly lower than that in the healthy control group; HMGB1 levels showed no obvious differences. In the KD group, HRG levels were negatively correlated with white blood cell and neutrophil counts. In the poor responders and responders groups, a tendency for a decrease in HRG and HMGB1 levels, respectively, was observed from pretreatment to post-treatment. Conclusions HRG and HMGB1 are related to the pathogenesis of KD; low HRG and high HMGB1 levels cause resistance against KD treatment.