ISSN 2997-1036
International Journal of Hematology | Vol. 11, No. 1, January 2020 | pp. 1–8
DOI: 10.46882/2020/IJH/000121
Original Article
Title: Clinical relevance of serum soluble CD14 and ferritin kinetics as early markers of macrophage activation syndrome in systemic juvenile idiopathic arthritis
Names of Authors: A. I. Ibrahim¹, C. D. Balogun², E. O. Ojo³
Authors’ Affiliations: ¹Department of Haematology, Ahmadu Bello University, Zaria, Nigeria; ²Department of Paediatrics, University of Ilorin, Ilorin, Nigeria; ³Department of Chemical Pathology, Ladoke Akintola University of Technology, Ogbomoso, Nigeria
Abstract: Macrophage activation syndrome is a hyperinflammatory, life-threatening complication of systemic autoimmune conditions characterized by cytopenias, multi-organ failure, and extreme hyperferritinemia. This prospective cohort study evaluated the diagnostic accuracy of monitoring serum soluble CD14 (sCD14) relative to hyperferritinemia kinetics in 36 pediatric patients with active systemic juvenile idiopathic arthritis suspected of developing macrophage activation syndrome. Serum soluble CD14 levels were quantified using a standardized enzyme-linked immunosorbent assay. Mean serum ferritin concentrations were significantly elevated in patients with confirmed macrophage activation syndrome (18,450 ± 4,200 ng/ml). Soluble CD14 concentrations were also markedly elevated, with a mean value of 14.6 ± 3.2 μg/ml compared to 2.1 ± 0.5 μg/ml in active disease controls without macrophage activation (P < 0.001). Soluble CD14 levels correlated strongly with falling platelet counts and elevated serum triglycerides (r = 0.62, P < 0.01). Receiver operating characteristic curve analysis established that a soluble CD14 threshold above 6.8 μg/ml yielded a diagnostic sensitivity of 91.6% and a specificity of 88.8%. Measuring soluble CD14 serves as a highly specific biomarker for active macrophage activation, helping differentiate this hyperinflammatory storm from typical underlying disease flare-ups.
Keywords: Macrophage activation syndrome, ferritin, soluble CD14, hemophagocytosis, juvenile idiopathic arthritis
Manuscript Timeline: Received: October 14, 2019; Revised: November 20, 2019; Accepted: December 08, 2019; Published: January 14, 2020