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Focal Segmental Glomerulosclerosis and Its Pathophysiology

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Proteinuria: Basic Mechanisms, Pathophysiology and Clinical Relevance

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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.

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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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  1. Division of Renal Diseases and Hypertension, University of Colorado Denver, 12700, E 19th Avenue, C281, Aurora, CO, 80045, USA

    James Dylewski & Judith Blaine

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  1. James Dylewski
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  2. Judith Blaine
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Correspondence to James Dylewski .

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    Judith Blaine

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Dylewski, J., Blaine, J. (2016). Focal Segmental Glomerulosclerosis and Its Pathophysiology. In: Blaine, J. (eds) Proteinuria: Basic Mechanisms, Pathophysiology and Clinical Relevance. Springer, Cham. https://doi.org/10.1007/978-3-319-43359-2_7

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