系统性硬皮病患者的成纤维细胞抗体与烯醇化酶结合，并与间质性肺病相关。

Antifibroblast antibodies from systemic sclerosis patients bind to {alpha}-enolase and are associated with interstitial lung disease.

机构信息

Paris Descartes University, UPRES EA, France.

出版信息

Ann Rheum Dis. 2010 Feb;69(2):428-33. doi: 10.1136/ard.2008.104299. Epub 2009 Mar 16.

DOI:10.1136/ard.2008.104299

PMID:19293162

Abstract

OBJECTIVE

To identify target antigens of antifibroblast antibodies (AFA) in systemic sclerosis (SSc) patients.

PATIENTS AND METHODS

In the first part, sera from 24 SSc patients (12 with pulmonary arterial hypertension (PAH) and 12 without) and 36 idiopathic PAH patients, tested in pooled sera for groups of three, were compared with a sera pool from 14 healthy controls (HC). Serum IgG reactivity was analysed by the use of a two-dimensional electrophoresis and immunoblotting technique with normal human fibroblasts antigens. In the second part, serum IgG reactivity for two groups: 158 SSc, 67 idiopathic PAH and 100 HC; and 35 SSc and 50 HC was tested against alpha-enolase from Saccharomyces cerevisiae and recombinant human (rHu) alpha-enolase, respectively, on ELISA.

RESULTS

In the first part, alpha-enolase was identified as a main target antigen of AFA from SSc patients. In the second part, 37/158 (23%) SSc patients, 6/67 (9%) idiopathic PAH patients and 4/100 (4%) HC (p<0.001) had anti-S cerevisiae alpha-enolase antibodies; 12/35 (34%) SSc patients and 3/50 (6%) HC had anti-rHu alpha-enolase antibodies (p = 0.001). In SSc, the presence of anti-S cerevisiae alpha-enolase antibodies was associated with interstitial lung disease (ILD), decreased total lung capacity (73.2% vs 89.7%; p<0.001) and diffusion capacity for carbon monoxide (47.4% vs 62.3%; p<0.001), and antitopoisomerase 1 antibodies (46% vs 21%; p = 0.005) but not anticentromere antibodies (11% vs 34%; p = 0.006). Results were similar with rHu alpha-enolase testing.

CONCLUSION

In SSc, AFA recognise alpha-enolase and are associated with ILD and antitopoisomerase antibodies.

摘要

目的

鉴定系统性硬化症（SSc）患者抗成纤维细胞抗体（AFA）的靶抗原。

方法

在第一部分中，将 24 例 SSc 患者（12 例伴有肺动脉高压（PAH）和 12 例不伴有）和 36 例特发性 PAH 患者的血清与 14 例健康对照者（HC）的血清池进行分组比较。血清 IgG 反应性通过二维电泳和免疫印迹技术与正常人成纤维细胞抗原进行分析。在第二部分中，用酶联免疫吸附试验（ELISA）检测 158 例 SSc、67 例特发性 PAH 和 100 例 HC 患者血清 IgG 对酿酒酵母α-烯醇酶和重组人（rHu）α-烯醇酶的反应性，以及 35 例 SSc 和 50 例 HC 患者血清 IgG 的反应性。

结果

在第一部分中，α-烯醇酶被鉴定为 SSc 患者 AFA 的主要靶抗原。在第二部分中，37/158（23%）例 SSc 患者、6/67（9%）例特发性 PAH 患者和 4/100（4%）例 HC（p<0.001）患者具有抗酿酒酵母α-烯醇酶抗体；12/35（34%）例 SSc 患者和 3/50（6%）例 HC 患者具有抗 rHuα-烯醇酶抗体（p = 0.001）。在 SSc 中，抗酿酒酵母α-烯醇酶抗体的存在与间质性肺病（ILD）、总肺容量下降（73.2% vs 89.7%；p<0.001）和一氧化碳弥散量降低（47.4% vs 62.3%；p<0.001）以及抗拓扑异构酶 1 抗体（46% vs 21%；p = 0.005）相关，但与抗着丝点抗体无关（11% vs 34%；p = 0.006）。使用 rHuα-烯醇酶检测的结果相似。