Neutrophil activation biomarker pentraxin 3 for diagnosis and monitoring of macrophage activation syndrome occurrence in adult-onset Still's disease

• DOI: 10.1016/j.jaut.2024.103182
• Publication Date: 2024-03-08T23:38:14Z
• Authors: Dehao Zhu; Longfang Chen; Jianfen Meng; Mengyan Wang; Yuning Ma; Xia Chen; Yu Xiao; Da Yi; Hui Shi; Yue Sun; Honglei Liu; Xiaobing Cheng; Yutong Su; Junna Ye; Huihui Chi; Zhuochao Zhou; Chengde Yang; Jialin Teng; Jinchao Jia; Qiongyi Hu
• Keywords: PTX3; Macrophage Activation Syndrome; Adult-onset Still's disease; S100A8

Macrophage activation syndrome (MAS) is a potentially fatal consequence of adult-onset Still's disease (AOSD), driven by cytokine storm. Efficient early diagnosis AOSD-associated MAS requires sensitive and specific biomarker. In this study, we demonstrated that pentraxin 3 (PTX3), an acute phase protein, was associated with AOSD activity served as biomarker for AOSD-MAS. PTX3 levels were significantly increased in patients compared to other autoimmune diseases healthy controls. Plasma showed positive correlations inflammatory markers, the systemic score HScore. active MAS, higher those non-AOSD haemophagocytic lymphohistiocytosis (HLH) patients. Moreover, PTX3's area under curve value distinguishing exceeded soluble interleukin-2 receptor, ferritin C-reactive protein. Furthermore, plasma circulating NET-DNA levels. To fully understand underlying mechanism prompting AOSD-MAS progression, discovered neutrophils exhibited enhanced NET formation mitogen-activated protein kinases (MAPK) pathway upon stimulation. More importantly, PTX3-induced effectively dampened MAPK inhibitors. These findings collectively revealed has favorable correlation may serve potential differentiate MAS. Additionally, induces release via pathway, suggesting pathogenic role