Department of Medicine Solna, Respiratory Medicine Unit, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.
Respir Med. 2011 Feb;105(2):282-91. doi: 10.1016/j.rmed.2010.11.018. Epub 2010 Dec 10.
Sarcoidosis is an inflammatory multisystemic granulomatous disease of unknown aetiology commonly affecting the lungs, and pulmonary fibrosis often develops in chronic sarcoidosis. It has been suggested that Wnt (Wingless/integrated)-signaling has a role in inflammatory and fibrotic processes in the lungs, but its role in sarcoidosis has not been investigated. We hypothesised that Wnts secreted from T cells or other inflammatory cells have a role in the pathogenesis of sarcoidosis.
Brush biopsies and bronchoalveolar lavage (BAL) were obtained through bronchoscopy from healthy controls (n = 18) and patients with sarcoidosis (n = 48). Semi-quantitative RT-PCR, electrophoretic mobility shift assay (EMSA) and immunocytochemistry were performed to analyse Wnt expression and activation of the Wnt-signal transducer β-catenin.
Altered expression of Wnt5A, Wnt7A and Wnt7B mRNA in BAL cells was observed, as well as an increased activation of β-catenin, measured by EMSA and confirmed with immunocytochemistry, in resident lung cells from patients with sarcoidosis. More pronounced changes in Wnt expression were seen with advancing disease stage. Thus, by three independent methods, we have found evidence of increased pulmonary Wnt-activation in sarcoidosis.
In the lungs of patients with sarcoidosis there is a previously unappreciated increased Wnt-signal activation that could contribute to the inflammatory processes.
结节病是一种病因不明的炎症性多系统肉芽肿性疾病,常累及肺部,慢性结节病常发展为肺纤维化。有研究表明,Wnt(无翅型整合素)信号在肺部的炎症和纤维化过程中起作用,但它在结节病中的作用尚未得到研究。我们假设 T 细胞或其他炎症细胞分泌的 Wnts 在结节病的发病机制中起作用。
通过支气管镜从健康对照者(n=18)和结节病患者(n=48)中获取刷检和支气管肺泡灌洗液(BAL)。采用半定量 RT-PCR、电泳迁移率变动分析(EMSA)和免疫细胞化学分析 Wnt 表达和 Wnt 信号转导物β-连环蛋白的激活。
观察到 BAL 细胞中 Wnt5A、Wnt7A 和 Wnt7B mRNA 的表达改变,以及通过 EMSA 测量和免疫细胞化学证实的 resident 肺细胞中β-连环蛋白的激活增加,在结节病患者中。随着疾病阶段的进展,Wnt 表达的变化更为明显。因此,我们通过三种独立的方法发现了结节病中肺 Wnt 激活增加的证据。
在结节病患者的肺部,存在以前未被认识到的 Wnt 信号激活增加,这可能有助于炎症过程。