The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor α-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis
The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor α-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis
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DOI：
10.1002/art.22416
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发表时间：
2007-02-01
影响因子：
--
通讯作者：
Grom, Alexei A.
中科院分区：
文献类型：
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作者：
Bleesing, Jack;Prada, Anne;Grom, Alexei A.
Objective.巨噬细胞活化综合征的特征在于由T细胞和噬血细胞巨噬细胞过度扩增驱动的压倒性炎症反应。可溶性白细胞介素-2受体α（sIL-2 R α）和可溶性CD 163（sCD 163）的水平可分别反映T细胞和巨噬细胞的活化和扩增程度。本研究旨在探讨血清sIL-2 Ra和sCD 163在幼年特发性关节炎（JIA）并发急性巨噬细胞活化综合征中的诊断价值。应用酶联免疫吸附试验（ELISA）检测了7例急性巨噬细胞活化综合征合并系统性JIA患者和16例初发系统性JIA患者血清sIL-2 Ra和sCD 163水平。该结果与巨噬细胞活化综合征的临床特征相关，包括铁蛋白水平。巨噬细胞活化综合征患者的sIL-2 R α水平中位数为19，646 pg/ml（四分位距[IQR] 18，128），而全身性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sCD 163的中位水平为23，000 ng/ml（IQR 14，191），而全身性JIA患者为5，480 ng/ml（IQR 2，635）（P = 0.017）。在16例系统性JIA患者中，5例血清sIL-2 Ra或sCD 163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动性，与血红蛋白水平降低（P = 0.11）、血小板计数降低和铁蛋白水平显著升高（P = 0.02）的趋势相关。这5例患者中有2例在几个月后出现明显的巨噬细胞活化综合征。sIL-2 Ra和sCD 163水平是巨噬细胞活化综合征有希望的诊断标志物。它们还可以帮助识别亚临床巨噬细胞活化综合征患者。
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 a (sIL-2R alpha) 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-2Ra and sCD163 in diagnosing acute macrophage activation syndrome complicating systemic juvenile idiopathic arthritis (JIA).Methods. Enzyme-linked immunosorbent assay was used to assess sIL-2Ra 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-2R alpha 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-2Ra 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-2Ra and sCD163 are promising diagnostic markers for macrophage activation syndrome. They may also help identify patients with subclinical macrophage activation syndrome.