Pharmacologic therapies for idiopathic pulmonary fibrosis, past and future.
Pharmacologic therapies for idiopathic pulmonary fibrosis, past and future.
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DOI：
10.3109/07853890.2014.991751
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发表时间：
2015-03
影响因子：
4.4
通讯作者：
Veeraraghavan S
中科院分区：
文献类型：
--
作者：
Staitieh BS;Renzoni EA;Veeraraghavan S
特发性肺纤维化(IPF)是一种原因不明的严重进行性肺纤维化疾病，在美国约有15万名患者受到影响。它的中位生存期为两到三年，但临床病程可能因患者而异。目前还没有针对IPF的既定治疗方法，但最近通过IPFnet等组织和学术界与业界的合作伙伴关系在协调临床试验方面取得的进展使人们能够更深入地研究这种相对罕见的疾病。从历史上看，IPF的默认治疗方法是泼尼松、N-乙酰半胱氨酸和硫唑嘌呤的联合治疗，但最近的试验表明，这种方案实际上增加了死亡率。近年来，从长椅到床边的大量工作从根本上改变了我们对IPF潜在分子机制的理解。较新的方法，特别是那些涉及针对特定途径的单抗的方法，在该领域引起了极大的兴奋，最近关于吡非尼酮和9tedanib等疗法的临床试验预示着靶向IPF疗法的一个新纪元。
Idiopathic pulmonary fibrosis (IPF) is a severe, progressive fibrotic disease of the lung of unknown etiology that affects approximately 150,000 patients in the United States. It carries a median survival of two to three years, but clinical course can vary markedly from patient to patient. There has been no established treatment for IPF, but recent advances in coordinated clinical trials through groups such as IPFnet and academia-industry partnerships have allowed this relatively rare disease to be studied in much greater depth. Historically, the default therapy for IPF was a combination of prednisone, N-acetylcysteine, and azathioprine, but recent trials have shown that this regimen actually increases mortality. An enormous body of work in recent years, spanning the bench to the bedside, has radically altered our understanding of the molecular mechanisms underlying IPF. Newer modalities, particularly those involving monoclonal antibodies targeted at specific pathways known to contribute to the fibrotic process, have generated a great deal of excitement in the field, and recent clinical trials on therapies such as pirfenidone and nintedanib herald a new era in targeted IPF therapies.