肺纤维化的治疗进展。
摘要
特发性肺纤维化（IPF）和进展性肺纤维化（PPF）的药物治疗正在从两种已确立的抗纤维化药物，向更广泛的治疗研发管线演变。目前采用尼达尼布和吡非尼酮的标准治疗可减缓用力肺活量的下降，但不能阻止疾病进展，且长期使用可能因耐受性问题而受限。…
正文 待译
Pharmacological treatment of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) is evolving from two established antifibrotic agents towards a broader pipeline of therapies. Current standard treatment with nintedanib and pirfenidone slows the decline in forced vital capacity, but does not halt disease progression, and long-term use may be limited by tolerability issues. This review summarises major profibrotic mechanisms relevant to drug development and discusses therapies in advanced or emerging clinical programmes, including the recently FDA-approved nerandomilast, positive results from the phase 3 trial of inhaled treprostinil, as well as promising mid-stage, pathway-directed agents targeting a broad range of profibrotic signalling pathways. We also discuss how negative or terminated programmes highlight recurring limitations related to efficacy, safety, trial duration, endpoint selection, and patient heterogeneity. Finally, we outline current shifts in trial design and outcome assessment, including increasing emphasis on patient-centred and composite endpoints alongside physiological measures, and discuss the rationale for add-on and combination approaches in future antifibrotic therapy for IPF and PPF.
English
Pharmacological treatment of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) is evolving from two established antifibrotic agents towards a broader pipeline of therapies. Current standard treatment with nintedanib and pirfenidone slows the decline in forced vital capacity, but does not halt disease progression, and long-term use may be limited by tolerability issues.…