Abstract：
OBJECTIVE: Progressive pulmonary fibrosis (PPF) is a newly recognised clinical phenotype of interstitial lung diseases in the 2022 interstitial pulmonary fibrosis (IPF) guidelines. This category is based entirely on clinical and radiological factors, and the background histopathology is unknown. Our objective was to investigate the histopathological characteristics of PPF and to examine the correlation between usual interstitial pneumonia (UIP) and prognosis in this new disease type. We hypothesised that the presence of UIP-like fibrosis predicts patients\' survival in PPF cases.
RESULTS: We selected 201 cases fulfilling the clinical criteria of PPF from case archives. Cases diagnosed as IPF by a multidisciplinary team were excluded. Whole slide images were evaluated by three pathologists who were blinded to clinical and radiological data. We measured areas of UIP-like fibrosis and calculated what percentage of the total lesion area they occupied. The presence of focal UIP-like fibrosis amounting to 10% or more of the lesion area was seen in 148 (73.6%), 168 (83.6%) and 165 (82.1%) cases for each pathologist, respectively. Agreement of the recognition of UIP-like fibrosis in PPF cases was above κ = 0.6 between all pairs. Survival analysis showed that the presence of focal UIP-like fibrosis correlated with worsened survival under all parameters tested (P < 0.001).
CONCLUSIONS: The presence of UIP-like fibrosis is a core pathological feature of clinical PPF, and its presence within diseased areas is associated with poorer prognosis. This study highlights the importance of considering the presence of focal UIP-like fibrosis in the evaluation and management of PPF.
RESULTS: We selected 201 cases fulfilling the clinical criteria of PPF from case archives. Cases diagnosed as IPF by a multidisciplinary team were excluded. Whole slide images were evaluated by three pathologists who were blinded to clinical and radiological data. We measured areas of UIP-like fibrosis and calculated what percentage of the total lesion area they occupied. The presence of focal UIP-like fibrosis amounting to 10% or more of the lesion area was seen in 148 (73.6%), 168 (83.6%) and 165 (82.1%) cases for each pathologist, respectively. Agreement of the recognition of UIP-like fibrosis in PPF cases was above κ = 0.6 between all pairs. Survival analysis showed that the presence of focal UIP-like fibrosis correlated with worsened survival under all parameters tested (P < 0.001).
CONCLUSIONS: The presence of UIP-like fibrosis is a core pathological feature of clinical PPF, and its presence within diseased areas is associated with poorer prognosis. This study highlights the importance of considering the presence of focal UIP-like fibrosis in the evaluation and management of PPF.
摘要：
目的：进行性肺纤维化（PPF）是2022年间质性肺纤维化（IPF）指南中新近确认的间质性肺疾病的临床表型。此类别完全基于临床和放射学因素，背景组织病理学未知。我们的目的是研究PPF的组织病理学特征，并检查这种新疾病类型的常见间质性肺炎（UIP）与预后之间的相关性。我们假设UIP样纤维化的存在可以预测PPF病例中患者的生存率。
结果：我们从病例档案中选择了201例符合PPF临床标准的病例。由多学科小组诊断为IPF的病例被排除。由三名对临床和放射学数据不知情的病理学家评估整个幻灯片图像。我们测量了UIP样纤维化的面积，并计算了它们占据的总病变面积的百分比。局灶性UIP样纤维化的存在占病变面积的10％或更多，见148（73.6％），每个病理学家168例（83.6%）和165例（82.1%），分别。在PPF病例中识别UIP样纤维化的一致性在所有配对之间高于κ=0.6。生存分析显示，在所有测试参数下，局灶性UIP样纤维化的存在与生存恶化相关（P<0.001）。
结论：UIP样纤维化的存在是临床PPF的核心病理特征，并且它在患病区域内的存在与预后较差有关。这项研究强调了在PPF的评估和管理中考虑局灶性UIP样纤维化的重要性。
结果：我们从病例档案中选择了201例符合PPF临床标准的病例。由多学科小组诊断为IPF的病例被排除。由三名对临床和放射学数据不知情的病理学家评估整个幻灯片图像。我们测量了UIP样纤维化的面积，并计算了它们占据的总病变面积的百分比。局灶性UIP样纤维化的存在占病变面积的10％或更多，见148（73.6％），每个病理学家168例（83.6%）和165例（82.1%），分别。在PPF病例中识别UIP样纤维化的一致性在所有配对之间高于κ=0.6。生存分析显示，在所有测试参数下，局灶性UIP样纤维化的存在与生存恶化相关（P<0.001）。
结论：UIP样纤维化的存在是临床PPF的核心病理特征，并且它在患病区域内的存在与预后较差有关。这项研究强调了在PPF的评估和管理中考虑局灶性UIP样纤维化的重要性。
