Dallos Tomáš, Heiland Gisela Ruiz, Strehl Johanna, Karonitsch Thomas, Gross Wolfgang L, Moosig Frank, Holl-Ulrich Constanze, Distler Jörg H W, Manger Bernhard, Schett Georg, Zwerina Jochen
University of Erlangen-Nuremberg, Erlangen, Germany; Comenius University in Bratislava, Bratislava, Slovakia.
Arthritis Rheum. 2010 Nov;62(11):3496-503. doi: 10.1002/art.27678.
Churg-Strauss syndrome (CSS) is a Th2-mediated systemic vasculitis characterized by eosinophilic infiltration, blood eosinophilia, and high IgE levels. CCL17/thymus and activation-regulated chemokine (TARC) is a chemokine responsible for the recruitment of Th2 cells. This study was undertaken to explore a possible role of CCL17/TARC in CSS.
CCL17/TARC levels in serum from patients with active or inactive CSS, hypereosinophilic syndrome, systemic small-vessel vasculitis other than CSS, other types of eosinophilia, and healthy controls were determined by enzyme-linked immunosorbent assay. Biopsy samples of affected tissue from CSS patients were examined by immunohistochemical staining for Th2 infiltration and CCL17/TARC expression.
Serum CCL17/TARC levels were significantly elevated in CSS patients with active disease (mean ± SEM 1,122.0 ± 422.7 pg/ml) compared with controls (220.6 ± 27.9 pg/ml) and patients with inactive disease (388.9 ± 72.6 pg/ml) (P < 0.001 and P < 0.05, respectively). These levels correlated with the clinical disease course of CSS and with absolute eosinophil counts as well as IgE levels. Infiltrating Th2 cells in active CSS lesions were evidenced by CD294 staining. CCL17/TARC in the affected tissue of CSS patients was readily identified by immunohistochemical analysis. Elevated CCL17/TARC levels were also noted in patients with hypereosinophilic syndrome (794.5 ± 294.8 pg/ml) and other disorders associated with eosinophilia (1,096.0 ± 345.3 pg/ml) (both P < 0.005 versus controls).
CCL17/TARC may contribute to CSS pathogenesis by recruitment of Th2 cells into affected tissue. Serum CCL17/TARC levels reflect disease activity, and further studies to validate its use as an activity marker in CSS are warranted.
变应性肉芽肿性血管炎（CSS）是一种由Th2介导的系统性血管炎，其特征为嗜酸性粒细胞浸润、血液嗜酸性粒细胞增多以及IgE水平升高。CCL17/胸腺和活化调节趋化因子（TARC）是一种负责募集Th2细胞的趋化因子。本研究旨在探讨CCL17/TARC在CSS中的可能作用。
采用酶联免疫吸附测定法测定活动期或非活动期CSS患者、高嗜酸性粒细胞综合征患者、CSS以外的系统性小血管炎患者、其他类型嗜酸性粒细胞增多症患者以及健康对照者血清中的CCL17/TARC水平。对CSS患者受累组织的活检样本进行免疫组织化学染色，以检测Th2浸润和CCL17/TARC表达。
与对照组（220.6±27.9 pg/ml）和非活动期疾病患者（388.9±72.6 pg/ml）相比，活动期CSS患者血清CCL17/TARC水平显著升高（平均值±标准误为1,122.0±422.7 pg/ml）（分别为P<0.001和P<0.05）。这些水平与CSS的临床病程、绝对嗜酸性粒细胞计数以及IgE水平相关。通过CD294染色证实活动期CSS病变中有Th2细胞浸润。通过免疫组织化学分析很容易在CSS患者的受累组织中鉴定出CCL17/TARC。高嗜酸性粒细胞综合征患者（794.5±294.8 pg/ml）和其他与嗜酸性粒细胞增多相关的疾病患者（1,096.0±345.3 pg/ml）的CCL17/TARC水平也升高（与对照组相比，两者均为P<0.005）。
CCL17/TARC可能通过将Th2细胞募集到受累组织中而促进CSS的发病机制。血清CCL17/TARC水平反映疾病活动度，有必要进行进一步研究以验证其作为CSS活动标志物的用途。
