Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown etiology and pathogenic mechanisms. From an etiopathogenic point of view, alveolar macrophages play a key role in accumulation of fibroblasts and deposition of collagen and extracellular matrix by releasing specific cytokines and inflammatory mediators. IPF seems to be also associated with circulating fibrocytes, which might be involved with an abnormal pulmonary vascular repair and remodeling. Based on its hypothesized pathologic mechanisms, anti-inflammatory, anti-fibrotic and immunosuppressive therapies are often used. For these reasons, Interferon-g (IFN-g) has been used to exploit its activity on macrophages and fibroblasts. The aim of this study was to investigate the response to corticosteroids and/or IFN-g 1b treatments based on pulmonary function tests and on inflammatory cytokine patterns of expression on bronchoalveolar lavage (BAL), at baseline and during and after the therapies. Unlike previous studies, we analyzed a period of therapy longer than 1 year. Our results demonstrated the effectiveness of IFN-γ in a group of IPF patients in whom the treatment was prolonged for over a year. These data suggest a positive role of IFN-γ; treatment in patients in the initial stage of the disease.

译文

:特发性肺纤维化(IPF)是一种病因和致病机理未知的慢性肺部疾病。从致病性的观点来看,肺泡巨噬细胞通过释放特定的细胞因子和炎性介质,在成纤维细胞的积累以及胶原蛋白和细胞外基质的沉积中起关键作用。 IPF似乎也与循环纤维细胞有关,可能与异常的肺血管修复和重塑有关。基于其假设的病理机制,通常使用抗炎,抗纤维化和免疫抑制疗法。由于这些原因,干扰素-g(IFN-g)已被用来开发其对巨噬细胞和成纤维细胞的活性。这项研究的目的是基于基线,治疗期间和治疗后,根据肺功能测试和支气管肺泡灌洗(BAL)的炎症性细胞因子表达模式,研究对皮质类固醇和/或IFN-g 1b治疗的反应。与以前的研究不同,我们分析了治疗时间超过1年的情况。我们的结果证明了IFN-γ在一组IPF患者中的有效性,这些患者的治疗时间延长了一年以上。这些数据表明IFN-γ具有积极作用。疾病初期患者的治疗。

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