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Pulmonary Fibrosis

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Front. Med. | doi: 10.3389/fmed.2018.00043

The Role of Immune and Inflammatory Cells in Idiopathic Pulmonary Fibrosis

  • 1Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale School of Medicine, Yale University, United States

The contribution of the immune system to Idiopathic Pulmonary Fibrosis (IPF) remains poorly understood. While most sources agree that IPF does not result from a primary immunopathogenic mechanism, evidence gleaned from animal modeling and human studies suggests that innate and adaptive immune processes can orchestrate existing fibrotic responses. This review will synthesize the available data regarding the complex role of professional immune cells in IPF. The role of innate immune populations such as monocytes, macrophages, myeloid suppressor cells, and innate lymphoid cells will be discussed, as will the activation of these cells via pathogen associated molecular patterns derived from invading or commensural microbes, and danger associated molecular patterns derived from injured cells and tissues. The contribution of adaptive immune responses driven by T-helper cells and B cells will be reviewed as well. Each form of immune activation will be discussed in the context of its relationship to environmental and genetic factors, disease outcomes, and potential therapies. We conclude with discussion of unanswered questions and opportunities for future study in this area.

Keywords: innate immunity, Adaptive Immunity, Macrophages, Lymphocytes, Fibroproliferation

Received: 10 Sep 2017; Accepted: 06 Feb 2018.

Edited by:

Argyrios Tzouvelekis, Alexander Fleming Biomedical Sciences Research Center, Greece

Reviewed by:

Michael A. O'Reilly, University of Rochester, United States
Gabriela Leuschner, Ludwig-Maximilians-Universität München, Germany  

Copyright: © 2018 Desai, Winkler, Minasyan and Herzog. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: MD, PhD. Erica Herzog, Yale School of Medicine, Yale University, Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, 300 Cedar Street TAC 441S, New Haven, 06520-8057, Connecticut, United States,