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Idiopathic pulmonary fibrosis and a role for autoimmunity
Journal article   Peer reviewed

Idiopathic pulmonary fibrosis and a role for autoimmunity

G.F. Hoyne, H. Elliott, S.E. Mutsaers and C.M. Prêle
Immunology & Cell Biology, Vol.95(7), pp.577-583
2017
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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.

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Collaboration types
Domestic collaboration
Citation topics
1 Clinical & Life Sciences
1.208 Vasculitis & Autoimmune Disorders
1.208.1262 Idiopathic Pulmonary Fibrosis
Web Of Science research areas
Cell Biology
Immunology
ESI research areas
Immunology
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