Serum Cytokine Profiling Differentiates Underlying Diseases in Cytokine Storm Syndrome.
Objective: Cytokine storm syndrome (CSS), commonly associated with hemophagocytic lymphohistiocytosis (HLH), is a fatal hyperinflammatory syndrome. Differentiating the underlying diseases responsible for CSS is essential for timely therapeutic decisions. This study explored the clinical usefulness o...
| Published in: | Arthritis & Rheumatology Vol. 78; no. 2; pp. 463 - 475 |
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| Main Authors: | , , , , , , , , , , , , , , , , , , , |
| Format: | research tables/charts Journal Article |
| Published: |
Wiley-Blackwell
Feb2026
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| Online Access: | View this record in EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=194234226&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 194234226 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 23265191 H06R jtl: Arthritis & Rheumatology issn: 23265191 maglogo: N pubinfo: dt: Feb2026 vid: 78 iid: 2 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 194234226 189633741 194234226 194234226 10.1002/art.43349 194234226 ppf: 463 ppct: 12 formats: tig: atl: Serum Cytokine Profiling Differentiates Underlying Diseases in Cytokine Storm Syndrome. aug: au: Kaneko, Shuya Hatano, Maho Shimbo, Asami Miyaoka, Futaba Irabu, Hitoshi Akutsu, Yuko Hayashi, Yuko Mizuta, Mao Nakagishi, Yasuo Akamine, Keiji Iwata, Naomi Furuno, Kenji Tanaka, Takayuki Ueno, Kazuyuki Fujita, Shuhei Yokoyama, Koji Minato, Toshinori Izawa, Kazushi Yasumi, Takahiro Matsubayashi, Tadashi affil: Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Institute of Science Tokyo, Tokyo, Japan sug: subj: Cytokine Release Syndrome Etiology Cytokine Release Syndrome Diagnosis Cytokines Blood Classification Algorithms Macrophage Activation Syndrome Hemophagocytic Lymphohistiocytosis Diagnosis, Differential Human Male Female Infant, Newborn Infant Child, Preschool Child Adolescence Adult Middle Age Aged Aged, 80 and Over Japan Multicenter Studies Nonexperimental Studies Enzyme-Linked Immunosorbent Assay Immunoassay Retrospective Design Record Review Medical Records Tumor Necrosis Factor Interleukins Intracellular Signaling Peptides and Proteins ROC Curve Sensitivity and Specificity Predictive Value of Tests Kruskal-Wallis Test Mann-Whitney U Test Paired T-Tests Pearson's Correlation Coefficient Descriptive Statistics Data Analysis Software Cytokine Release Syndrome Classification Cytokine Release Syndrome Therapy Immune System Infant, Newborn: birth-1 month Infant: 1-23 months Child, Preschool: 2-5 years Child: 6-12 years Adolescent: 13-18 years Adult: 19-44 years Middle Aged: 45-64 years Aged: 65+ years Aged, 80 & over Male Female ab: Objective: Cytokine storm syndrome (CSS), commonly associated with hemophagocytic lymphohistiocytosis (HLH), is a fatal hyperinflammatory syndrome. Differentiating the underlying diseases responsible for CSS is essential for timely therapeutic decisions. This study explored the clinical usefulness of serum cytokine profiling in distinguishing underlying diseases in patients with CSS. Methods: Serum samples were collected from 143 adult and pediatric patients with CSS and 22 healthy controls. The cohort included patients with various diagnoses of primary and secondary HLH and Kawasaki disease (KD)‐like hyperinflammatory syndromes. Serum levels of 48 cytokines were analyzed in 97 patients using a bead‐based multiplex immunoassay (Luminex assay). Serum levels of interferon alpha (IFN‐α), interleukin‐18 (IL‐18), IL‐6, CXCL9, and soluble tumor necrosis factor receptor II (sTNF‐RII) were measured in 165 participants using enzyme‐linked immunosorbent assay (ELISA). Results: Luminex assay categorized patients with CSS into five clusters based on serum cytokine patterns. ELISA revealed distinct cytokine patterns, wherein patients with histiocytic necrotizing lymphadenitis‐associated HLH and systemic lupus erythematosus‐associated macrophage activation syndrome (MAS) showed elevated IFN‐α; systemic juvenile idiopathic arthritis–associated and adult‐onset Still's disease–associated MAS, X‐linked inhibitor of apoptosis protein deficiency with HLH, and NLRC4‐associated autoinflammatory disorder exhibited higher IL‐18 levels. Additionally, KD shock syndrome had higher IL‐6 levels than the other groups. CXCL9 was significantly elevated in patients with virus‐associated HLH, familial HLH, malignant lymphoma‐associated HLH, and KD‐MAS. Multisystem inflammatory syndrome in children and toxic shock syndrome also showed moderate elevations of CXCL9 and IL‐6 levels. Conclusion: Serum cytokine profiling effectively differentiates CSS subtypes, facilitating better diagnosis and personalized treatment strategies based on specific disease backgrounds. pubtype: Academic Journal doctype: research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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