Focal segmental glomerulosclerosis

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Last reviewed: June 2019

Last updated: June 2019

Summary

Caused by an injury to podocytes in the renal glomeruli.

May be primary (idiopathic) due to an unknown cause or secondary to an underlying condition, such as HIV, obesity, medications, and a maladaptive response to decreased renal mass.

May be asymptomatic or present with nephrotic syndrome.

First-line therapy in primary focal segmental glomerulosclerosis with nephrotic-range proteinuria is corticosteroid therapy. Secondary form requires treatment of the underlying cause.

Corticosteroid-dependent and corticosteroid-resistant patients require treatment with other immunosuppressants such as cyclosporine.

Prognosis is extremely variable and depends on the amount of proteinuria, the level of plasma creatinine, the morphologic subtype, the degree of renal fibrosis, and the response to therapy.

Definition

Focal segmental glomerulosclerosis (FSGS) is a chronic pathologic process caused by injury to podocytes in the renal glomeruli. It manifests initially with proteinuria, which progresses to nephrotic syndrome and ultimately to end-stage renal failure.

History and exam

Key diagnostic factors

edema
foamy urine
hypertension
Muehrcke lines
xanthelasma and xanthomata

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Other diagnostic factors

body mass index 95th percentile or greater
lymphadenopathy
skin rash
oral lesions
nephrectomy scar

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Risk factors

male sex
black race
family history of FSGS
heroin abuse
use of known causative medications
chronic viral infection
solitary kidney
pre-existing renal disease
kidney transplant
acute renal ischemia
obesity

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Diagnostic investigations

1st investigations to order

serum BUN
creatinine
GFR
urinalysis with microscopy
urine protein-to-creatinine ratio
24-hour urine collection for protein
serum albumin
serum lipid profile

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Investigations to consider

serum HIV enzyme-linked immunosorbent assay
CD4 count and viral load studies
parvovirus DNA polymerase chain reaction (PCR)
cytomegalovirus DNA PCR
hepatitis B and C serologies
antinuclear antibody, anti-double-stranded DNA
serum and urine protein electrophoresis
renal biopsy

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Treatment algorithm

ACUTE

primary FSGS

secondary FSGS

Contributors

Authors VIEW ALL 

Manish Suneja, MD, FASN, FACP

Professor

Department of Internal Medicine

Division of Nephrology

University of Iowa Hospitals and Clinics

Iowa City

IA

Disclosures

MS receives royalties from McGraw-Hill as an editor of the book DeGowin's Diagnostic Examination.

Acknowledgements

Dr Manish Suneja would like to gratefully acknowledge Dr Divya Movva, a previous contributor to this topic.

Peer reviewers VIEW ALL 

Catherine Clase, BA, MB, MSC, FRCPC

Associate Professor

Nephrologist

McMaster University

Hamilton

Ontario

Canada

Disclosures

CC declares that she has no competing interests.

Alain Meyrier, MD

Professor of Medicine

Universite Paris-Descartes

Hopital Europeen Georges Pompidou

Paris

France

Disclosures

AM is an author of a reference cited in this topic.

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