Systemic Inflammatory Response Syndrome, a Form of Cytokine Storm Associated with Adult-onset Still's Disease

Abstract

Abstract Objective This study aimed to analyse data regarding the clinical characteristics and outcomes of adult-onset Still's disease (AOSD) patients with systemic inflammatory response syndrome (SIRS). Methods A retrospective analysis of 72 AOSD patients at the First Affiliated Hospital of Zhengzhou University was conducted comparing the clinical characteristics, treatment protocols, and outcomes of patients with SIRS and macrophage activation syndrome (MAS). Results This study included 15 AOSD patients with SIRS (12 women) and 57 with MAS (47 women). The mean (SD) age of the patients with SIRS was 30.1 (14.2) years, and that of patients with MAS was 34.6 (14.9) years. Clinical features of these patients included fever, hepatosplenomegaly, polyserositis, and shock. There were statistically significant differences in leukocyte counts, platelets, albumin, lactate dehydrogenase, ferritin, erythrocyte sedimentation rate, and C-reactive protein values between the two groups. Patients with SIRS had lower HScore scores than patients with MAS (171 [156, 192] vs. 216 [178, 248], P = 0.02). Patients were treated with glucocorticoids (13 SIRS, 55 MAS), cyclosporine (7 SIRS, 49 MAS), intravenous immunoglobulin (8 SIRS, 30MAS), etoposide (5 SIRS, 34 MAS), and tocilizumab (2 SIRS, 21 MAS). The mortality rate of patients with SIRS (40%) was higher than that of patients with MAS (1.8%, P < 0.001). Conclusions SIRS is a rare but often fatal form of cytokine storm caused by persistent AOSD activity. Early treatment with immunosuppressants, etoposide, and IL-1 and IL-6 inhibitors is necessary for these patients.