CRP and sCD25 help distinguish between adult‐onset Still's disease and HLH

• Published in: European journal of haematology 2024-11, Vol.113 (5), p.576-583
• Main Authors: Beckett, Madelaine, Spaner, Caroline, Goubran, Mariam, Wade, John, Avina‐Zubieta, Juan Antonio, Setiadi, Audi, Tucker, Lori, Shojania, Kam, Au, Sheila, Mattman, Andre, Lee, Agnes Y. Y., Fajgenbaum, David C., Chen, Luke Y. C.
• Format: Article
• Language: English
• Subjects: Adult; Aged; autoinflammatory; Biomarkers - blood; C-Reactive Protein - analysis; C-Reactive Protein - metabolism; CD25 antigen; Cell activation; Cytokine storm; Diagnosis, Differential; Female; Ferritin; Ferritins - blood; Fibrin Fibrinogen Degradation Products - analysis; Fibrin Fibrinogen Degradation Products - metabolism; Hematology; Histiocytosis; HLH; Humans; Inflammatory diseases; Interleukin 6; Interleukin-2 Receptor alpha Subunit - blood; Juvenile rheumatoid arthritis; Lymphocytes T; Lymphocytosis; Lymphohistiocytosis, Hemophagocytic - blood; Lymphohistiocytosis, Hemophagocytic - diagnosis; Macrophages; Male; Middle Aged; Retrospective Studies; ROC Curve; soluble IL‐2 receptor; Still's disease; Still's Disease, Adult-Onset - blood; Still's Disease, Adult-Onset - diagnosis
• ISSN: 0902-4441; 1600-0609; 1600-0609
• DOI: 10.1111/ejh.14267

Objective Adult‐onset Still's disease (AOSD) and secondary hemophagocytic lymphohistiocytosis (sHLH) are both hyperferritinemic cytokine storm syndromes that can be difficult to distinguish from each other in hospitalized patients. The objective of this study was to compare the inflammatory markers ferritin, D‐dimer, C‐reactive protein (CRP), and soluble CD25 (sCD25) in patients with AOSD and sHLH. These four markers were chosen as they are widely available and represent different aspects of inflammatory diseases: macrophage activation (ferritin); endothelialopathy (D‐dimer); interleukin‐1/interleukin‐6/tumour necrosis factor elevation (CRP) and T cell activation (sCD25). Methods This was a single‐center retrospective study. Patients diagnosed by the Hematology service at Vancouver General Hospital for AOSD or sHLH from 2009 to 2023 were included. Results There were 16 AOSD and 44 sHLH patients identified. Ferritin was lower in AOSD than HLH (median 11 360 μg/L vs. 29 020 μg/L, p = .01) while D‐dimer was not significantly different (median 5310 mg/L FEU vs. 7000 mg/L FEU, p = .3). CRP was higher (median 168 mg/L vs. 71 mg/L, p 130 mg/L and sCD25