可溶性CD163和可溶性白细胞介素-2受体α链在巨噬细胞活化综合征及未经治疗的新发系统性幼年特发性关节炎中的诊断意义

The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis.

作者信息

Bleesing Jack, Prada Anne, Siegel David M, Villanueva Joyce, Olson Judyann, Ilowite Norman T, Brunner Hermine I, Griffin Thomas, Graham Thomas B, Sherry David D, Passo Murray H, Ramanan Athimalaipet V, Filipovich Alexandra, Grom Alexei A

机构信息

Children's Hospital Medical Center, Cincinnati, Ohio 45229, USA.

出版信息

Arthritis Rheum. 2007 Mar;56(3):965-71. doi: 10.1002/art.22416.

DOI:10.1002/art.22416

PMID:17328073

Abstract

OBJECTIVE

Macrophage activation syndrome is characterized by an overwhelming inflammatory reaction driven by excessive expansion of T cells and hemophagocytic macrophages. Levels of soluble interleukin-2 receptor alpha (sIL-2Ralpha) and soluble CD163 (sCD163) may reflect the degree of activation and expansion of T cells and macrophages, respectively. This study was undertaken to assess the value of serum sIL-2Ralpha and sCD163 in diagnosing acute macrophage activation syndrome complicating systemic juvenile idiopathic arthritis (JIA).

METHODS

Enzyme-linked immunosorbent assay was used to assess sIL-2Ralpha and sCD163 levels in sera from 7 patients with acute macrophage activation syndrome complicating systemic JIA and 16 patients with untreated new-onset systemic JIA. The results were correlated with clinical features of established macrophage activation syndrome, including ferritin levels.

RESULTS

The median level of sIL-2Ralpha in the patients with macrophage activation syndrome was 19,646 pg/ml (interquartile range [IQR] 18,128), compared with 3,787 pg/ml (IQR 3,762) in patients with systemic JIA (P = 0.003). Similarly, the median level of sCD163 in patients with macrophage activation syndrome was 23,000 ng/ml (IQR 14,191), compared with 5,480 ng/ml (IQR 2,635) in patients with systemic JIA (P = 0.017). In 5 of 16 patients with systemic JIA, serum levels of sIL-2Ralpha or sCD163 were comparable with those in patients with acute macrophage activation syndrome. These patients had high inflammatory activity associated with a trend toward lower hemoglobin levels (P = 0.11), lower platelet counts, and significantly higher ferritin levels (P = 0.02). Two of these 5 patients developed overt macrophage activation syndrome several months later.

CONCLUSION

Levels of sIL-2Ralpha and sCD163 are promising diagnostic markers for macrophage activation syndrome. They may also help identify patients with subclinical macrophage activation syndrome.

摘要

目的

巨噬细胞活化综合征的特征是由T细胞和噬血细胞性巨噬细胞过度扩增驱动的强烈炎症反应。可溶性白细胞介素-2受体α（sIL-2Rα）和可溶性CD163（sCD163）水平可能分别反映T细胞和巨噬细胞的活化及扩增程度。本研究旨在评估血清sIL-2Rα和sCD163在诊断并发系统性幼年特发性关节炎（JIA）的急性巨噬细胞活化综合征中的价值。

方法

采用酶联免疫吸附测定法评估7例并发系统性JIA的急性巨噬细胞活化综合征患者和16例未经治疗的新发系统性JIA患者血清中的sIL-2Rα和sCD163水平。将结果与已确诊的巨噬细胞活化综合征的临床特征（包括铁蛋白水平）进行关联分析。

结果

巨噬细胞活化综合征患者的sIL-2Rα中位水平为19,646 pg/ml（四分位间距[IQR] 18,128），而系统性JIA患者为3,787 pg/ml（IQR 3,762）（P = 0.003）。同样，巨噬细胞活化综合征患者的sCD163中位水平为23,000 ng/ml（IQR 14,191），而系统性JIA患者为5,480 ng/ml（IQR 2,635）（P = 0.017）。在16例系统性JIA患者中，有5例的血清sIL-2Rα或sCD163水平与急性巨噬细胞活化综合征患者相当。这些患者具有高炎症活性，伴有血红蛋白水平降低趋势（P = 0.11）、血小板计数降低以及铁蛋白水平显著升高（P = 0.02）。这5例患者中有2例在数月后发展为明显的巨噬细胞活化综合征。