Medimmune UK, Cambridge, UK.
Curr Opin Rheumatol. 2012 Nov;24(6):656-62. doi: 10.1097/BOR.0b013e3283588de4.
Pulmonary fibrosis is a devastating disease that affects millions of people worldwide. Among the most common forms of lung fibrosis are idiopathic pulmonary fibrosis (IPF) and scleroderma-related interstitial lung disease (SSc-ILD). Despite a wealth of literature regarding each of these diseases, studies that directly compare IPF and SSc-ILD are rare.
This review compares the salient features of IPF and SSc-ILD. Clinical presentation and demographics will be presented, along with the newly released radiographic and pathologic criteria for IPF. Evolving concepts of pathogenesis including the role of structural cell injury, the pathogenic role of macrophages and lymphocytes, and the origin of fibroblasts are described. We conclude with new developments in the search for predictive biomarkers of disease progression, such as markers of epithelial injury, lymphocyte subsets, and circulating fibrocytes, will be presented. We conclude with a discussion of the results of recent clinical trials.
It is found that despite differences in clinical presentation and response to treatment, similarities are noted in proposed pathogenesis and putative biomarkers. It is hoped that this information will lead to studies aimed at understanding the factors driving these difficult to treat and often deadly diseases.
肺纤维化是一种严重的疾病，影响着全球数百万人。在最常见的肺纤维化形式中，特发性肺纤维化（IPF）和硬皮病相关间质性肺疾病（SSc-ILD）。尽管关于这两种疾病的文献很多，但直接比较 IPF 和 SSc-ILD 的研究却很少。
本综述比较了 IPF 和 SSc-ILD 的显著特征。将介绍临床表现和人口统计学特征，以及新发布的 IPF 放射学和病理学标准。描述了发病机制的新进展，包括结构细胞损伤的作用、巨噬细胞和淋巴细胞的致病作用以及成纤维细胞的起源。我们将介绍疾病进展预测生物标志物的新进展，例如上皮损伤标志物、淋巴细胞亚群和循环成纤维细胞。最后讨论了最近临床试验的结果。
尽管临床表现和治疗反应存在差异，但在发病机制和潜在生物标志物方面存在相似之处。希望这些信息将导致针对这些难以治疗且通常致命疾病的驱动因素的研究。
