Progressive pulmonary fibrosis: a state-of-the-art review.

A significant proportion of patients with fibrosing ILDs other than IPF will develop a progressive phenotype comparable to IPF.

除了IPF之外，相当一部分纤维化间质性肺疾病患者将发展出与IPF相当的进行性表型。

Progression occurs despite conventional treatment which, depending on the underlying condition, may include close monitoring, antigen eviction, glucocorticoids, immunosuppressive therapy, and pulmonary rehabilitation.

尽管进行了常规治疗，疾病仍会进展，这可能包括密切监测、抗原驱逐、糖皮质激素、免疫抑制治疗和肺康复等，具体取决于基础疾病。

Progressive pulmonary fibrosis (PPF) has a disease course similar to IPF, with worsening respiratory symptoms, decline in lung function, impairment of quality of life, and premature death.

进行性肺纤维化（PPF）的疾病进程与特发性肺纤维化（IPF）相似，表现为呼吸症状恶化、肺功能下降、生活质量受损和过早死亡。

In 2019, a phase III trial demonstrated that treatment with the tyrosine kinase inhibitor nintedanib halves disease progression as measured by a decline in forced vital capacity over one year, and contributed to the validation of the PPF concept.

2019年的一项III期试验表明，酪氨酸激酶抑制剂尼替单抗治疗可使疾病进展减半，这是通过一年内用力肺活量下降来衡量的，并有助于PPF概念的验证。

In the 2022 international clinical practice guideline, nintedanib received a conditional recommendation to treat patients with PPF.

在2022年的国际临床实践指南中，尼替单抗获得了有条件推荐，用于治疗PPF患者。

In 2025, it was demonstrated that the phosphodiesterase 4B inhibitor nerandomilast further reduces disease progression in patients with PPF.

2025年的研究表明，磷酸二酯酶4B抑制剂nerandomilast进一步降低了PPF患者的疾病进展。

Other smaller trials have suggested that pirfenidone may also benefit patients with PPF.

其他一些小型试验表明，吡非尼酮也可能对PPF患者有益。

Several newer compounds are currently being developed for both IPF and PPF.

目前有几种新的化合物正在为特发性肺纤维化(IPF)和继发性肺纤维化(PPF)开发。

As a diagnosis of PPF identifies patients with fibrotic disease progression, early identification is warranted for the timely initiation of antifibrotic therapy, consideration of lung transplantation if eligible, and holistic care.

由于PPF的诊断能够识别出纤维化疾病进展的患者，因此早期识别对于及时开始抗纤维化治疗、考虑符合条件的肺移植以及全面护理至关重要。