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Focal Segmental Glomerulosclerosis and Its Pathophysiology

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Abstract

Focal segmental glomerulosclerosis, or FSGS, is a proteinuric kidney disease named based on the pattern of scarring seen on a kidney biopsy which can result from many different causes/reasons. FSGS has become the most common primary glomerular disease leading to end-stage kidney disease in the United States and is observed in approximately one-third of biopsies done for idiopathic nephrotic syndrome (Appel and D’Agati, Comprehensive clinical nephrology, 5th edn, 2015, pp. 218–230; D’Agati et al., N Engl J Med 365(25):2398–2411, 2011; Korbet, J Am Soc Nephrol 23(11):1769–1776, 2012). There is still much we do not know about the disease process as a whole, yet our understanding of how a similar final pattern of injury can develop from a multitude of etiologies has expanded. Due to the number of causes for FSGS and our ever expanding knowledge of this disorder, determining the reason for the disease and providing appropriate treatment remains a challenge.

In this chapter, we will discuss the known causes for this disease and how they are believed to lead to a similar pattern of injury. We will also define the histological types of FSGS and how they can help determine the cause of the injury. Finally, we will describe the clinical features of this disease and discuss potential treatment options.

Keywords

FSGS Proteinuria Genetic mutations Histologic subtype Immunosuppression 

Abbreviations

ACEi

Angiotensin converting enzyme inhibitor

APOL1

Apolipoprotein L1

ARB

Angiotensin receptor blocker

BP

Blood pressure

CLC-1

Cardiotrophin-like cytokine 1

CNI

Calcineurin inhibitor

COQ

Coenzyme Q

ESRD

End stage renal disease

FSGS

Focal segmental glomerulosclerosis

HAN

Heroin associated nephropathy

HIVAN

Human immunodeficiency virus associated nephropathy

KDIGO

Kidney disease improving global outcomes

MAP

Mean arterial pressure

MMF

Mycophenolate mofetil

mTOR

Mechanistic target of rapamycin

RAAS

Renin angiotensin aldosterone system

suPAR

Soluble urokinase plasminogen activator receptor

TGF-β

Transformimg growth factor beta

TNFα

Tumor necrosis factor alpha

TRPC6

Transient receptor potential cation channel 6

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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Division of Renal Diseases and HypertensionUniversity of Colorado DenverAuroraUSA

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