从疾病特点看特发性肺纤维化尽早干预的重要性

Importance of early intervention of idiopathic pulmonary fibrosis in terms of disease characteristics

特发性肺纤维化(IPF)是一类肺功能进行性下降的间质性肺疾病,整体预后差且疾病进展难以预测,即使是轻度患者也可能因急性加重导致病情快速进展。目前超过50%的IPF患者未开展规范的抗纤维化治疗。现有研究证明,对于疾病不同阶段和高度疑似的IPF患者在接受抗纤维化治疗后均能在延缓肺功能下降、降低急性加重风险上获得同等获益,提示所有IPF患者,即使是病情较轻或高度疑似病例,应该在诊断后尽早启动抗纤维化治疗,以最大程度延缓疾病进展、改善预后。

Idiopathic pulmonary fibrosis （IPF） is an interstitial pulmonary disease with progressive decline in lung function and poor prognosis. It is difficult to predict the progression oflPF, and even mild cases may progress rapidly following an acute exacerbation. Currentlyless than 50% IPF patients are under standard anti-fibrosis therapy. Mounnting evidences have demoustmted that IPF patients at different stages and even those highly-suspected cases can share similar clinical benefits from anti-fibrosis therapy regarding reduction of lung ftuaction decline and exacerbation risk. These data suggest that all IPF patients, including those with preserved lung function and highly-suspected cases, should initiate anti-fibrosis therapy once diagnosis is made, in order to delay disease progression and improve prognosis.