title: [Case of systemic-onset juvenile idiopathic arthritis complicated with sepsis --usefulness of cytokine profile for the differentiation between macrophage activation syndrome and sepsis].
abstract: The differential diagnosis of macrophage activation syndrome (MAS) and sepsis must be considered in the clinical course of systemic-onset juvenile idiopathic arthritis (s-JIA) with sudden onset of high-grade fever and abnormal laboratory findings, including leukocytopenia, thrombocytopenia, and coagulopathy. In this report, we describe the case of a 17-month-old girl diagnosed with s-JIA complicated with sepsis. Her serum interleukin (IL)-18 level was significantly elevated throughout the clinical course. Furthermore, compared to other MAS patients, she showed a significantly elevated serum IL-6 level and procalcitonin in sepsis. Therefore, our results suggest that a patient's cytokine profile may be a useful indicator of disease activity and may thus help in the differential diagnosis of sepsis and MAS in s-JIA.
authors.affiliation: Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University.
authors.forename: Tadafumi
authors.initials: T
authors.lastname: Yokoyama
authors.forename: Masaki
authors.initials: M
authors.lastname: Shimizu
authors.forename: Iku
authors.initials: I
authors.lastname: Ikeno
authors.forename: Yoko
authors.initials: Y
authors.lastname: Hashida
authors.forename: Syuhei
authors.initials: S
authors.lastname: Fujita
authors.forename: Shoetu
authors.initials: S
authors.lastname: Shimura
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Hatasaki
authors.forename: Naohisa
authors.initials: N
authors.lastname: Shintani
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yachie
mesh_terms: D001171:Arthritis, Juvenile / Q000150:complications* / Q000175:diagnosis*; D015415:Biomarkers / Q000097:blood; D002116:Calcitonin / Q000097:blood; D015740:Calcitonin Gene-Related Peptide; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D020382:Interleukin-18 / Q000097:blood*; D015850:Interleukin-6 / Q000097:blood*; D055501:Macrophage Activation Syndrome; D011498:Protein Precursors / Q000097:blood; D018805:Sepsis / Q000150:complications* / Q000175:diagnosis*
chemical_list: D015415:Biomarkers; C000606589:CALCA protein, human; D020382:Interleukin-18; D015850:Interleukin-6; D011498:Protein Precursors; D002116:Calcitonin; D015740:Calcitonin Gene-Related Peptide