What's in a name? That which we call IPF, by any other name would act the same
What's in a name? That which we call IPF, by any other name would act the same
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DOI：
10.1183/13993003.00692-2018
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发表时间：
2018-05-01
影响因子：
24.3
通讯作者：
Thannickal, Victor J.
中科院分区：
文献类型：
--
作者：
Wells, Athol U.;Brown, Kevin K.;Thannickal, Victor J.
特发性肺纤维化（IPF）仍然是一种真正的特发性纤维化疾病，具有适度的遗传偏好和候选触发因素，但对非家族性病例中疾病的发展没有总体解释。自千年以来，术语的一致性促进了重大的临床和翻译进展。目前由术语“IPF”捕获的实体很可能在未来十年内通过“分裂”（分为对个体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病（具有共同的发病机制和IPF样疾病行为）“合并”而被彻底重新分类。从这个角度来看，我们总结了临床和发病机制的理由，重点放在“进行性纤维化表型”在未来的临床和转化研究。通过这种方式，我们有望满足患有不可阻挡的进行性纤维化疾病的非IPF患者的需求，这些患者目前因无法获得对IPF有效的药物而被剥夺了权利。在这方面，正在进行的非IPF进行性纤维化患者抗纤维化治疗试验可能具有高度影响力。如果有重大的概念变化，未来可能需要对IPF命名进行修订，但没有令人信服的理由，重新命名IPF的好处可能会被由此产生的混乱所抵消。
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 antifibrotic 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.