Unknown

Dataset Information

0

Immune dysregulation as a driver of idiopathic pulmonary fibrosis.


ABSTRACT: 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.

SUBMITTER: Shenderov K 

PROVIDER: S-EPMC7810481 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

altmetric image

Publications

Immune dysregulation as a driver of idiopathic pulmonary fibrosis.

Shenderov Kevin K   Collins Samuel L SL   Powell Jonathan D JD   Horton Maureen R MR  

The Journal of clinical investigation 20210101 2


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  ...[more]

Similar Datasets

| S-EPMC7461042 | biostudies-literature
| S-EPMC7498242 | biostudies-literature
| S-EPMC4226053 | biostudies-literature
| S-EPMC9297827 | biostudies-literature
| S-EPMC4861274 | biostudies-literature
| S-EPMC8120991 | biostudies-literature
| S-EPMC6831929 | biostudies-literature
2023-01-01 | GSE195770 | GEO
| PRJNA802122 | ENA
| S-EPMC9075032 | biostudies-literature