Immune dysregulation as a driver of idiopathic pulmonary fibrosis

被引：198

作者：

Shenderov, Kevin ^{[1
,2
]}

Collins, Samuel L. ^{[1
,2
]}

Powell, Jonathan D. ^{[2
]}

Horton, Maureen R. ^{[1
]}

机构：

[1] Johns Hopkins Univ, Sch Med, Dept Med, Div Pulm & Crit Care, 1830 E Monument St,5th Floor, Bakimore, MD 21205 USA

[2] Johns Hopkins Univ, Sch Med, Sidney Kimmel Comprehens Canc Res Ctr, Bloomberg Kimrsel Inst Canc Immunotherapy,Dept On, Baltimore, MD 21205 USA

来源：

JOURNAL OF CLINICAL INVESTIGATION | 2021年 / 131卷 / 02期

关键词：

GROWTH-FACTOR-BETA; ALTERNATIVE MACROPHAGE ACTIVATION; GENE-EXPRESSION PROFILES; ALVEOLAR MACROPHAGES; T-CELLS; BRONCHOALVEOLAR LAVAGE; DISEASE PROGRESSION; SYSTEMIC-SCLEROSIS; LUNG-DISEASE; BLEOMYCIN;

D O I：

10.1172/JCI143226

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

Idiopathic pulmonary fibrosis (IPF) affects hundreds of thousands of people worldwide, reducing their quality of life and leading to death from respiratory failure within years of diagnosis. Treatment options remain limited, with only two FDA-approved drugs available in the United States, neither of which reverse the lung damage caused by the disease or prolong the life of individuals with IPF. The only cure for IPF is lung transplantation. In this review, we discuss recent major advances in our understanding of the role of the immune system in IPF that have revealed immune dysregulation as a critical driver of disease pathophysiology. We also highlight ways in which an improved understanding of the immune system's role in IPF may enable the development of targeted immunomodulatory therapies that successfully halt or potentially even reverse lung fibrosis.

引用

页数：10

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