Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

被引：21

作者：

Shumar, John N. ^{[1
]}

Chandel, Abhimanyu ^{[1
]}

King, Christopher S. ^{[2
]}

机构：

[1] Walter Reed Natl Mil Med Ctr, Dept Pulm & Crit Care Med, Bethesda, MD 20814 USA

[2] Inova Fairfax Hosp, Adv Lung Dis & Transplant Program, Falls Church, VA 22042 USA

来源：

JOURNAL OF CLINICAL MEDICINE | 2021年 / 10卷 / 11期

关键词：

progressive fibrosing interstitial lung disease; pulmonary fibrosis; pirfenidone; nintedanib; interstitial lung disease; IPF; antifibrotics; IDIOPATHIC PULMONARY-FIBROSIS; OBSERVATIONAL COHORT; TELOMERE LENGTH; PIRFENIDONE; MECHANISMS; NINTEDANIB; INHIBITOR; DIAGNOSIS; SURVIVAL; EFFICACY;

D O I：

10.3390/jcm10112285

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Progressive fibrosing interstitial lung disease (PF-ILD) describes a phenotypic subset of interstitial lung diseases characterized by progressive, intractable lung fibrosis. PF-ILD is separate from, but has radiographic, histopathologic, and clinical similarities to idiopathic pulmonary fibrosis. Two antifibrotic medications, nintedanib and pirfenidone, have been approved for use in patients with idiopathic pulmonary fibrosis. Recently completed randomized controlled trials have demonstrated the clinical efficacy of antifibrotic therapy in patients with PF-ILD. The validation of efficacy of antifibrotic therapy in PF-ILD has changed the treatment landscape for all of the fibrotic lung diseases, providing a new treatment pathway and opening the door for combined antifibrotic and immunosuppressant drug therapy to address both the fibrotic and inflammatory components of ILD characterized by mixed pathophysiologic pathways.

引用

页数：12

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