Idiopathic pulmonary fibrosis and a role for autoimmunity

Gerard F Hoyne; Hannah Elliott; Steven E Mutsaers; Cecilia Prele

doi:10.1038/icb.2017.22

Idiopathic pulmonary fibrosis and a role for autoimmunity

Gerard F Hoyne, Hannah Elliott, Steven E Mutsaers, Cecilia Prele

Research output: Contribution to journal › Article › peer-review

55 Citations (Scopus)

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is typically associated with extensive and progressive fibrosis, and is fatal and has limited treatment options. Characteristically IPF patients display large lymphocyte aggregates composed of CD3(+) T cells and CD20(+) B cells within the lung tissue that are located near sites of active fibrosis. In addition, IPF patients can have autoantibodies to a range of host antigens, suggesting a breakdown in immunological tolerance. In this review, we examine the role of T and B cells in IPF pathogenesis and discuss how loss of self-tolerance to lung-specific proteins could exacerbate disease progression in IPF. We discuss what these results mean in terms of future prospects for immunotherapy of IPF.Immunology and Cell Biology advance online publication, 25 April 2017; doi:10.1038/icb.2017.22.

Original language	English
Pages (from-to)	577–583
Number of pages	7
Journal	Immunology and Cell Biology
Volume	95
Issue number	7
Early online date	25 Apr 2017
DOIs	https://doi.org/10.1038/icb.2017.22
Publication status	Published - 1 Aug 2017

Access to Document

10.1038/icb.2017.22

Cite this

@article{4cbe874fb26c433b8e8f0a6e4a0de5c6,

title = "Idiopathic pulmonary fibrosis and a role for autoimmunity",

abstract = "Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is typically associated with extensive and progressive fibrosis, and is fatal and has limited treatment options. Characteristically IPF patients display large lymphocyte aggregates composed of CD3(+) T cells and CD20(+) B cells within the lung tissue that are located near sites of active fibrosis. In addition, IPF patients can have autoantibodies to a range of host antigens, suggesting a breakdown in immunological tolerance. In this review, we examine the role of T and B cells in IPF pathogenesis and discuss how loss of self-tolerance to lung-specific proteins could exacerbate disease progression in IPF. We discuss what these results mean in terms of future prospects for immunotherapy of IPF.Immunology and Cell Biology advance online publication, 25 April 2017; doi:10.1038/icb.2017.22.",

keywords = "Journal Article, Review",

author = "Hoyne, {Gerard F} and Hannah Elliott and Mutsaers, {Steven E} and Cecilia Prele",

year = "2017",

month = aug,

day = "1",

doi = "10.1038/icb.2017.22",

language = "English",

volume = "95",

pages = "577–583",

journal = "Immunology and Cell Biology",

issn = "0818-9641",

publisher = "Nature Publishing Group - Macmillan Publishers",

number = "7",

}

TY - JOUR

T1 - Idiopathic pulmonary fibrosis and a role for autoimmunity

AU - Hoyne, Gerard F

AU - Elliott, Hannah

AU - Mutsaers, Steven E

AU - Prele, Cecilia

PY - 2017/8/1

Y1 - 2017/8/1

N2 - Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is typically associated with extensive and progressive fibrosis, and is fatal and has limited treatment options. Characteristically IPF patients display large lymphocyte aggregates composed of CD3(+) T cells and CD20(+) B cells within the lung tissue that are located near sites of active fibrosis. In addition, IPF patients can have autoantibodies to a range of host antigens, suggesting a breakdown in immunological tolerance. In this review, we examine the role of T and B cells in IPF pathogenesis and discuss how loss of self-tolerance to lung-specific proteins could exacerbate disease progression in IPF. We discuss what these results mean in terms of future prospects for immunotherapy of IPF.Immunology and Cell Biology advance online publication, 25 April 2017; doi:10.1038/icb.2017.22.

AB - Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is typically associated with extensive and progressive fibrosis, and is fatal and has limited treatment options. Characteristically IPF patients display large lymphocyte aggregates composed of CD3(+) T cells and CD20(+) B cells within the lung tissue that are located near sites of active fibrosis. In addition, IPF patients can have autoantibodies to a range of host antigens, suggesting a breakdown in immunological tolerance. In this review, we examine the role of T and B cells in IPF pathogenesis and discuss how loss of self-tolerance to lung-specific proteins could exacerbate disease progression in IPF. We discuss what these results mean in terms of future prospects for immunotherapy of IPF.Immunology and Cell Biology advance online publication, 25 April 2017; doi:10.1038/icb.2017.22.

KW - Journal Article

KW - Review

U2 - 10.1038/icb.2017.22

DO - 10.1038/icb.2017.22

M3 - Article

C2 - 28356570

SN - 0818-9641

VL - 95

SP - 577

EP - 583

JO - Immunology and Cell Biology

JF - Immunology and Cell Biology

IS - 7

ER -

Idiopathic pulmonary fibrosis and a role for autoimmunity

Abstract

Access to Document

Fingerprint

Cite this