Therapeutic Approach to Adult Fibrotic Lung Diseases.

Therapeutic Approach to Adult Fibrotic Lung Diseases.
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DOI:
10.1016/j.chest.2016.07.027
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发表时间:
2016-12
期刊:
影响因子:
9.6
通讯作者:
Strek ME
Strek ME
中科院分区:
医学1区
文献类型:
--
作者:
Adegunsoye A;Strek ME

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在间质性肺疾病 (ILD) 中,特发性肺纤维化 (IPF)、慢性过敏性肺炎和纤维化结缔组织病相关 ILD 的预后较差,呼吸衰竭和严重相关合并症会导致死亡。最近开发和批准的抗纤维化药物尼达尼布和吡非尼酮在临床试验中均降低了 IPF 患者肺功能下降的速度,这为改变与 IPF 相关的死亡率增加带来了希望。尽管慢性过敏性肺炎和结缔组织病相关 ILD 可能与炎症成分有关,但在治疗中使用免疫抑制剂的证据很大程度上仅限于回顾性研究。免疫抑制治疗在晚期纤维化中缺乏益处,因此也需要在这些类型的 ILD 中使用抗纤维化治疗进行严格的临床试验。纤维化 ILD 患者可能会受益于相关合并症(如肺动脉高压、胃食管反流和 OSA)的识别和治疗,这可能会改善患者的生活质量,在某些情况下甚至可以改善患者的生存率。由于早期评估可能会优化移植后的结果,因此 IPF 和其他形式的纤维化 ILD 患者应尽早进行肺移植评估。
Among the interstitial lung diseases (ILDs), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis, and fibrotic connective tissue disease-related ILD are associated with a worse prognosis, with death occurring as a result of both respiratory failure and serious associated comorbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF. Although chronic hypersensitivity pneumonitis and connective tissue disease related-ILD may be associated with an inflammatory component, the evidence for the use of immunosuppressive agents in their treatment is largely limited to retrospective studies. The lack of benefit of immunosuppressive therapy in advanced fibrosis argues for rigorous clinical trials using antifibrotic therapies in these types of ILD as well. Patients with fibrotic ILD may benefit from identification and management of associated comorbid conditions such as pulmonary hypertension, gastroesophageal reflux, and OSA, which may improve the quality of life and, in some cases, survival in affected individuals. Because early assessment may optimize posttransplantation outcomes, lung transplant evaluation should occur early in patients with IPF and those with other forms of fibrotic ILD.
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