This review provides an overview of literature addressing progressive massive fibrosis (PMF) from September 2009 to the present. Advances are described in understanding its pathophysiology, epidemiology of the occurrence of PMF and related conditions, the impact of PMF on pulmonary function, advances in imaging of PMF, and factors affecting progression of pneumoconiosis in dust-exposed workers to PMF. Basic advances in understanding the etiology of PMF are impeded by the lack of a well-accepted animal model for human PMF. Recent studies evaluating lung tissue samples and epidemiologic investigations support an important role for the silica component of coal mine dust in causing coal workers' pneumoconiosis and PMF in contemporary coal miners in the United States and for silica in causing silicosis and PMF in artificial stone workers throughout the world. Development of PMF is associated with substantial decline in pulmonary function relative to no disease or small opacity pneumoconiosis. In recent reports, computed tomography has had greater sensitivity for detecting PMF than chest x-ray. Magnetic resonance imaging shows promise in differentiating between PMF and lung cancer. Although PMF develops in dust-exposed workers without previously identified small opacity pneumoconiosis, the presence of small opacity pneumoconiosis increases the risk for progression to PMF, as does heavier dust exposure. Recent literature does not document any effective new treatments for PMF and new therapies to prevent and treat PMF are an important need.

本综述概述了从 2009 年 9 月至今涉及进行性大块纤维化 (PMF) 的文献。介绍了了解其病理生理学、PMF 发生和相关病症的流行病学、PMF 对肺功能的影响、PMF 影像学进展以及影响接触 PMF 的粉尘工人肺尘病进展的因素等方面的进展。由于缺乏公认的人类 PMF 动物模型，阻碍了对 PMF 病因学的基本了解。最近评估肺组织样本和流行病学调查的研究支持煤矿粉尘中的二氧化硅成分在导致煤工 美国当代煤矿工人的尘肺病和 PMF，以及全世界人造石工人因二氧化硅引起的矽肺病和 PMF。相对于无疾病或小混浊尘肺，PMF 的发展与肺功能的显着下降有关。在最近的报告中，计算机断层扫描检测 PMF 的灵敏度高于胸部 X 线检查。磁共振成像显示了区分 PMF 和肺癌的希望。尽管 PMF 在接触粉尘的工人中发展，之前没有发现小混浊性尘肺，但小混浊性尘肺的存在增加了进展为 PMF 的风险，较重的粉尘暴露也是如此。最近的文献没有记载任何有效的 PMF 新疗法，并且重要的是需要新疗法来预防和治疗 PMF。