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The Role of Adaptive Immunity in Idiopathic Pulmonary Fibrosis: Hiding in Plain Sight

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Idiopathic Pulmonary Fibrosis

Part of the book series: Respiratory Medicine ((RM,volume 9))

Abstract

Deleterious actions of antigen-activated T and B lymphocytes, the major cellular effectors of adaptive immune host defenses, underlie the pathogenesis of nearly every human fibroproliferative disease. Misdirected or especially intense and persistent adaptive immune responses can cause recurrent cellular injury and dysregulated tissue repair in other organs that are comparable to the lung abnormalities found in patients with idiopathic pulmonary fibrosis (IPF). This chapter includes a brief discussion of some relevant basic immunobiology to facilitate a better understanding of the mechanisms and typifying features of pathological adaptive immune responses. Subsequent sections then review findings of the numerous translational studies that implicate adaptive immunity in the development and progression of IPF. Many diseases caused by adaptive immune responses are, like IPF, refractory to treatment with nonspecific glucocorticoid regimens. However, patients with these other immunological disorders often benefit from treatment regimens that specifically target the causal inflammatory mechanism(s). The author believes that appreciation of the abnormal immune processes associated with IPF would justify trials of novel, mechanistically focused therapies that have the potential to benefit patients with this morbid and heretofore intractable lung disease.

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Acknowledgments

Supported in part by NIH grant: HL107172.

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  1. Department of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center, 628 NW MUH, 3459 Fifth Ave, Pittsburgh, PA, 15213, USA

    Steven R. Duncan M.D.

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  1. Dept. Internal Medicine, Sect. Allergy Pulmonary & Critical Care Med., University of Wisconsin School of Medicine & Public Health, Madison, Wisconsin, USA

    Keith C. Meyer

  2. Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia, USA

    Steven D. Nathan

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Duncan, S.R. (2014). The Role of Adaptive Immunity in Idiopathic Pulmonary Fibrosis: Hiding in Plain Sight. In: Meyer, K., Nathan, S. (eds) Idiopathic Pulmonary Fibrosis. Respiratory Medicine, vol 9. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-682-5_7

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