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The Role of Inflammation and Fibrosis in Cystic Kidney Disease

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Polycystic Kidney Disease

Abstract

Interstitial inflammation and fibrosis are among the most notable hallmarks of cystic kidney diseases. These biological processes represent key components of a complex multidirectional relationship that, together with abnormal injury and tissue repair, as well as renal tubular and vascular epithelial function, defines the pathobiology of renal cystic disease progression. This chapter provides a summary of key findings relevant to the role of inflammation and fibrosis in this complex relationship. It reviews major polycystic kidney disease (PKD)-associated inflammatory pathways, immune cell types and modulators of immune function, and their potential roles in the development of PKD therapeutics and identification of predictive biomarkers.

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Authors and Affiliations

  1. Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA

    James C. Harms MD & Michal Mrug MD

  2. Department of Cell, Developmental and Integrative Biology, University of Alabama at Birmingham, Birmingham, AL, USA

    Cheng Jack Song BS

  3. Department of Veterans Affairs Medical Center, Birmingham, AL, USA

    Michal Mrug MD

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Correspondence to Michal Mrug MD .

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Editors and Affiliations

  1. John Gammill Professor in Polycystic Kidney Disease, Chief, Section of Nephrology and Hypertension, Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA

    Benjamin D. Cowley, Jr.

  2. Federal Express Chair of Excellence, Chief, Division of Nephrology, Department of Pediatrics, Director, Tuberous Sclerosis Center of Excellence at Le Bonheur Children’s Hospital, Medical Director, Nephrology St. Jude Children’s Research Hospital, University of Tennessee Health Science Center, Children’s Foundation Research Center, Memphis, Tennessee, USA

    John J. Bissler

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Harms, J.C., Song, C.J., Mrug, M. (2018). The Role of Inflammation and Fibrosis in Cystic Kidney Disease. In: Cowley, Jr., B., Bissler, J. (eds) Polycystic Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-7784-0_6

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