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Am J Manag Care. 2024 Oct;30(7 Suppl):S122-S130. doi: 10.37765/ajmc.2024.89634.
The term "progressive pulmonary fibrosis" or "PPF" is generally used to describe progressive lung fibrosis in an individual with an interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis (IPF). Several sets of criteria have been proposed for the identification of PPF, most of which are based on a combination of a decline in forced vital capacity, worsening of respiratory symptoms, and increase in the extent of fibrosis on radiology. Although some risk factors for faster progression of fibrosing ILD have been identified, it remains challenging to predict which individuals will develop PPF. Close monitoring, including regular pulmonary function tests, is required to detect the earliest signs of worsening disease. PPF is associated with high rates of hospitalization and death. Management of PPF requires a multidisciplinary and multimodal approach, including pharmacological therapy and supportive care. Discussions about palliative care should begin at an early stage, individualized to the needs of the patient.
“进行性肺纤维化”或“PPF”一词通常用于描述患有特发性肺纤维化（IPF）以外的间质性肺疾病（ILD）的个体的进行性肺纤维化。已经提出了几套用于识别 PPF 的标准，其中大多数标准基于用力肺活量下降、呼吸症状恶化以及影像学上纤维化程度增加的组合。尽管已经确定了一些导致纤维化性ILD 更快进展的危险因素，但预测哪些个体将发展为 PPF 仍然具有挑战性。需要密切监测，包括定期进行肺功能测试，以检测疾病恶化的最早迹象。PPF 与高住院率和死亡率相关。PPF 的管理需要多学科和多模式方法，包括药物治疗和支持性护理。应该在早期开始讨论姑息治疗，并根据患者的需求进行个体化。
