Immune dysregulation as a driver of idiopathic pulmonary fibrosis
Kevin Shenderov,1,2 Samuel L. Collins,1,2 Jonathan D. Powell,2 and Maureen R. Horton1
1Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
2Bloomberg-Kimmel Institute for Cancer Immunotherapy, Sidney Kimmel Comprehensive Cancer Research Center, Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Address correspondence to: Maureen R. Horton, Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, 1830 E. Monument St., 5th Floor, Baltimore, Maryland 21205, USA. Phone: 410.955.4176; Eamil: mhorton2@jhmi.edu.
Authorship note: KS and SLC contributed equally to this work.
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1Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
2Bloomberg-Kimmel Institute for Cancer Immunotherapy, Sidney Kimmel Comprehensive Cancer Research Center, Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Address correspondence to: Maureen R. Horton, Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, 1830 E. Monument St., 5th Floor, Baltimore, Maryland 21205, USA. Phone: 410.955.4176; Eamil: mhorton2@jhmi.edu.
Authorship note: KS and SLC contributed equally to this work.
Find articles by Collins, S. in: JCI | PubMed | Google Scholar
1Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
2Bloomberg-Kimmel Institute for Cancer Immunotherapy, Sidney Kimmel Comprehensive Cancer Research Center, Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Address correspondence to: Maureen R. Horton, Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, 1830 E. Monument St., 5th Floor, Baltimore, Maryland 21205, USA. Phone: 410.955.4176; Eamil: mhorton2@jhmi.edu.
Authorship note: KS and SLC contributed equally to this work.
Find articles by Powell, J. in: JCI | PubMed | Google Scholar
1Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
2Bloomberg-Kimmel Institute for Cancer Immunotherapy, Sidney Kimmel Comprehensive Cancer Research Center, Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Address correspondence to: Maureen R. Horton, Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, 1830 E. Monument St., 5th Floor, Baltimore, Maryland 21205, USA. Phone: 410.955.4176; Eamil: mhorton2@jhmi.edu.
Authorship note: KS and SLC contributed equally to this work.
Find articles by Horton, M. in: JCI | PubMed | Google Scholar
Published January 19, 2021 - More info
J Clin Invest. 2021;131(2):e143226. https://doi.org/10.1172/JCI143226.
© 2021 American Society for Clinical Investigation
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.
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