Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.
National Jewish Health, Denver, CO, USA.
Eur Respir J. 2018 May 17;51(5). doi: 10.1183/13993003.00692-2018. Print 2018 May.
Idiopathic pulmonary fibrosis (IPF) remains a truly idiopathic fibrotic disease, with a modest genetic predilection and candidate triggers but no overall explanation for the development of disease in non-familial cases. Agreement on terminology has contributed to major clinical and translational advances since the millennium. It is likely that the entity currently captured by the term "IPF" will be radically reclassified over the next decade, either through "splitting" (into IPF subgroups responding selectively to individual disease-modifying agents) or through "lumping" of IPF with other forms of progressive fibrotic lung disease (with shared pathogenetic mechanisms and IPF-like disease behaviour). In this perspective, we summarise the clinical and pathogenetic justification for a focus on "the progressive fibrotic phenotype" in future clinical and translational research. By this means, we can hope to address the needs of non-IPF patients with inexorably progressive fibrotic disease, currently disenfranchised by lack of access to agents that are efficacious in IPF. In this regard, ongoing trials of anti-fibrotic therapies in non-IPF patients with progressive fibrosis may be highly influential. Future revision of IPF nomenclature may be warranted if there are major conceptual changes but without compelling justification, the benefits of renaming IPF are likely to be outweighed by the resulting confusion.
特发性肺纤维化（IPF）仍然是一种真正的特发性纤维化疾病，具有适度的遗传倾向和候选触发因素，但对于非家族性病例中疾病的发展尚无总体解释。自千禧年以来，术语的一致性促进了重大的临床和转化进展。目前被称为“IPF”的实体很可能在未来十年内通过“分裂”（分为对个别疾病修饰剂有选择性反应的 IPF 亚组）或通过将 IPF 与其他形式的进行性纤维化肺疾病“合并”（具有共同的发病机制和类似 IPF 的疾病行为）而得到彻底重新分类。从这个角度来看，我们总结了在未来的临床和转化研究中关注“进行性纤维化表型”的临床和发病机制依据。通过这种方式，我们可以希望满足目前因缺乏在 IPF 中有效的药物而被剥夺权利的进行性纤维化疾病非 IPF 患者的需求。在这方面，正在进行的针对进行性纤维化的非 IPF 患者的抗纤维化治疗试验可能具有重要影响。如果存在重大概念变化但没有令人信服的理由，则可能需要对 IPF 命名进行修订，但重新命名 IPF 的好处可能会被由此产生的混乱所抵消。
