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Flow Cytometric Identification of Fibrocytes in Scleroderma Lung Disease

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Autoimmunity

Part of the book series: Methods in Molecular Biology ((MIMB,volume 900))

Abstract

Scleroderma is an autoimmune disease characterized by the progressive and dysregulated accumulation of collagen in the skin and internal organs. Pulmonary complications including interstitial lung disease have emerged as the greatest cause of mortality in this disease. Because treatments are limited, new areas of investigation are sorely needed. An emerging area of interest in this field is a potential role for fibrocytes as biomarkers or mediators of disease. Fibrocytes are monocyte-derived mesenchymal progenitor cells that exhibit features of extracellular matrix production and wound contraction in addition to immunologic functions such as cytokine and chemokine production, antigen presentation, leukocyte trafficking, and modulation of angiogenesis. Fibrocytes could participate in the pathogenesis of scleroderma lung disease through any or all of these functions and may be useful biomarkers of disease activity. This chapter presents protocols that have been developed for the study of fibrocytes obtained from human circulation and tissues. Protocols for the quantification of fibrocytes in murine models also are described, along with discussion of common technical challenges. It is hoped that this information will allow further investigation of the role that fibrocytes might play in Scleroderma-related lung disease and perhaps lead to new areas of study in this difficult-to-treat and deadly disease.

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Acknowledgements

This work was supported in part by grants K08 HL079066 from the National Institutes of Health, the National Scleroderma Foundation, the American Thoracic Society, and funds from the Yale Department of Internal Medicine.

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Authors and Affiliations

  1. Section of Pulmonary and Critical Care Medicine, Department of Medicine, Yale University, New Haven, CT, USA

    Thomas M. Russell & Erica L. Herzog

  2. Yale University, New Haven, CT, USA

    Richard Bucala

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  1. Thomas M. Russell
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  2. Erica L. Herzog
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  3. Richard Bucala
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Correspondence to Richard Bucala .

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Editors and Affiliations

  1. College of Medicine, Division of Rheumatology, State University of New York, UMU, East Adams St. 750, Syracuse, 13210, New York, USA

    Andras Perl

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Russell, T.M., Herzog, E.L., Bucala, R. (2012). Flow Cytometric Identification of Fibrocytes in Scleroderma Lung Disease. In: Perl, A. (eds) Autoimmunity. Methods in Molecular Biology, vol 900. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-720-4_16

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  • DOI: https://doi.org/10.1007/978-1-60761-720-4_16

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  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-60761-719-8

  • Online ISBN: 978-1-60761-720-4

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