Eosinophil Cationic Protein (ECP), a predictive marker of bullous pemphigoid severity and outcome.

Eosinophil Cationic Protein (ECP), a predictive marker of bullous pemphigoid severity and outcome.
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DOI:
10.1038/s41598-017-04687-5
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发表时间:
2017-07-06
期刊:
影响因子:
4.6
通讯作者:
Pham BN
Pham BN
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Giusti D;Gatouillat G;Le Jan S;Plée J;Bernard P;Antonicelli F;Pham BN

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大疱性类天疱疮(BP)是一种罕见的炎症性自身免疫性大疱性皮肤病,其结果无法通过临床研究预测。嗜酸性粒细胞是BP中主要的免疫浸润细胞。然而,嗜酸性粒细胞激活时主要碱性蛋白(MBP)、嗜酸性粒细胞衍生神经毒素(EDN)和嗜酸性粒细胞阳离子蛋白(ECP)的释放尚未针对BP的发展进行评估。通过 ELISA 测定基线时 BP 患者的血清 (n = 61) 和疱液 (n = 20) 以及治疗 2 个月后的血清 (n = 41) 中的 MBP、EDN 和 ECP。与对照受试者 (n = 20) 相比,BP 患者在基线时的 MBP、EDN 和 ECP 血清浓度显着升高,表明嗜酸性粒细胞激活,但根据疾病严重程度或结果没有区别。疱液中的 EDN 和 ECP 值甚至更高(分别为 P < 0.01 和 P < 0.05),而 MBP 值较低(P < 0.001)。治疗 60 天后,持续缓解的 BP 患者 ECP 血清浓度下降,但后来复发的患者则没有下降(P < 0.05)。 ECP浓度至少降低12.8ng/mL可提供81%缓解的阳性预测值,表明ECP血清变异可能是对有复发风险的BP患者进行分层的有用生物标志物。
Bullous Pemphigoid (BP) is an inflammatory rare autoimmune bullous dermatosis, which outcome cannot be predicted through clinical investigations. Eosinophils are the main immune infiltrated cells in BP. However, the release of Major Basic Protein (MBP), Eosinophil Derived Neurotoxin (EDN), and Eosinophil Cationic Protein (ECP) upon eosinophil activation has still not been evaluated with respect to BP development. MBP, EDN and ECP were measured by ELISA in serum (n = 61) and blister fluid (n = 20) of patients with BP at baseline, and in serum after 2 months of treatment (n = 41). Eosinophil activation in BP patients was illustrated at baseline by significantly higher MBP, EDN and ECP serum concentrations as compared with control subjects (n = 20), but without distinction according to disease severity or outcome. EDN and ECP values were even higher in the blister fluids (P < 0.01 and P < 0.05, respectively), whereas MBP values were lower (P < 0.001). ECP serum concentration decreased after 60 days of treatment in BP patients with ongoing remission but not in patients who later relapsed (P < 0.05). A reduction of at least 12.8 ng/mL in ECP concentrations provided a positive predictive value for remission of 81%, showing that ECP serum variation could be a useful biomarker stratifying BP patients at risk of relapse.
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