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Abstract：
Histopathologic criteria of usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) were defined over the years and endorsed by leading organizations decades after Dr. Averill A. Liebow first coined the term UIP in the 1960s as a distinct pathologic pattern of fibrotic interstitial lung disease. Novel technology and recent research on interstitial lung diseases with genetic component shed light on molecular pathogenesis of UIP/IPF. Two antifibrotic agents introduced in the mid-2010s opened a new era of therapeutic approaches to UIP/IPF, albeit contentious issues regarding their efficacy, side effects, and costs. Recently, the concept of progressive pulmonary fibrosis was introduced to acknowledge additional types of progressive fibrosing interstitial lung diseases with the clinical and pathologic phenotypes comparable to those of UIP/IPF. Likewise, some authors have proposed a paradigm shift by considering UIP as a stand-alone diagnostic entity to encompass other fibrosing interstitial lung diseases that manifest a relentless progression as in IPF. These trends signal a pendulum moving toward the tendency of lumping diagnoses, which poses a risk of obscuring potentially important information crucial to both clinical and research purposes. Recent advances in whole slide imaging for digital pathology and artificial intelligence technology could offer an unprecedented opportunity to enhance histopathologic evaluation of interstitial lung diseases. However, current clinical practice trends of moving away from surgical lung biopsies in interstitial lung disease patients may become a limiting factor in this endeavor as it would be difficult to build a large histopathologic database with correlative clinical data required for artificial intelligence models.
摘要：
多年来，定义了常规间质性肺炎（UIP）/特发性肺纤维化（IPF）的组织病理学标准，并在AverillA.Liebow博士于1960年代首次将术语UIP作为纤维化间质性肺病的独特病理模式后几十年得到了领先组织的认可。具有遗传成分的间质性肺疾病的新技术和最新研究揭示了UIP/IPF的分子发病机制。2010年代中期引入的两种抗纤维化药物开启了UIP/IPF治疗方法的新时代，尽管关于它们的功效有争议，副作用,和成本。最近,引入进行性肺纤维化的概念是为了确认其他类型的进行性纤维化间质性肺病,其临床和病理表型与UIP/IPF相当.同样，一些作者提出了一种范式转变,将UIP视为独立的诊断实体,以涵盖其他纤维化间质性肺病,这些疾病表现出与IPF一样的持续进展.这些趋势标志着钟摆朝着集中诊断的趋势发展，这构成了模糊对临床和研究目的至关重要的潜在重要信息的风险。用于数字病理学和人工智能技术的全载玻片成像的最新进展可能为增强间质性肺疾病的组织病理学评估提供前所未有的机会。然而,目前的临床实践趋势是在间质性肺病患者中放弃外科肺活检可能成为这项工作中的限制因素,因为很难建立一个大型组织病理学数据库,其中包含人工智能模型所需的相关临床数据.
