U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Segmental glomerulosclerosis

MedGen UID:
83080
•Concept ID:
C0333497
•
Disease or Syndrome
Synonym: Focal sclerosis with hyalinosis
SNOMED CT: Segmental glomerulosclerosis (16104005)
 
HPO: HP:0033495

Definition

Segmental solidification of the glomerular tuft by increased extracellular matrix, causing glomerular capillary obliteration and can include hyalinosis, foam cells, hypertrophy of overlying glomerular epithelial cells, podocyte depletion, halo and adhesion of the tuft to the Bowman capsule. [from HPO]

Professional guidelines

PubMed

Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases.
Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan TM, Cook HT, Fervenza FC, Gibson KL, Glassock RJ, Jayne DRW, Jha V, Liew A, Liu ZH, Mejía-Vilet JM, Nester CM, Radhakrishnan J, Rave EM, Reich HN, Ronco P, Sanders JF, Sethi S, Suzuki Y, Tang SCW, Tesar V, Vivarelli M, Wetzels JFM, Lytvyn L, Craig JC, Tunnicliffe DJ, Howell M, Tonelli MA, Cheung M, Earley A, Floege J
Kidney Int 2021 Oct;100(4):753-779. doi: 10.1016/j.kint.2021.05.015. PMID: 34556300
Nephrotic syndrome in infants and children: pathophysiology and management.
Downie ML, Gallibois C, Parekh RS, Noone DG
Paediatr Int Child Health 2017 Nov;37(4):248-258. Epub 2017 Sep 15 doi: 10.1080/20469047.2017.1374003. PMID: 28914167
Diagnosis and Management of Nephrotic Syndrome in Adults.
Kodner C
Am Fam Physician 2016 Mar 15;93(6):479-85. PMID: 26977832

Recent clinical studies

Etiology

Nephrotic Syndrome.
Politano SA, Colbert GB, Hamiduzzaman N
Prim Care 2020 Dec;47(4):597-613. Epub 2020 Sep 26 doi: 10.1016/j.pop.2020.08.002. PMID: 33121631
Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective.
Shabaka A, Tato Ribera A, Fernández-Juárez G
Nephron 2020;144(9):413-427. Epub 2020 Jul 28 doi: 10.1159/000508099. PMID: 32721952
Nephrotic Syndrome.
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Focal Segmental Glomerulosclerosis.
Rosenberg AZ, Kopp JB
Clin J Am Soc Nephrol 2017 Mar 7;12(3):502-517. Epub 2017 Feb 27 doi: 10.2215/CJN.05960616. PMID: 28242845Free PMC Article
Causes and pathogenesis of focal segmental glomerulosclerosis.
Fogo AB
Nat Rev Nephrol 2015 Feb;11(2):76-87. Epub 2014 Dec 2 doi: 10.1038/nrneph.2014.216. PMID: 25447132Free PMC Article

Diagnosis

Therapeutic trials in adult FSGS: lessons learned and the road forward.
De Vriese AS, Wetzels JF, Glassock RJ, Sethi S, Fervenza FC
Nat Rev Nephrol 2021 Sep;17(9):619-630. Epub 2021 May 20 doi: 10.1038/s41581-021-00427-1. PMID: 34017116Free PMC Article
Nephrotic Syndrome.
Politano SA, Colbert GB, Hamiduzzaman N
Prim Care 2020 Dec;47(4):597-613. Epub 2020 Sep 26 doi: 10.1016/j.pop.2020.08.002. PMID: 33121631
Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective.
Shabaka A, Tato Ribera A, Fernández-Juárez G
Nephron 2020;144(9):413-427. Epub 2020 Jul 28 doi: 10.1159/000508099. PMID: 32721952
Nephrotic Syndrome.
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Glomerular diseases: FSGS.
Bose B, Cattran D; Toronto Glomerulonephritis Registry
Clin J Am Soc Nephrol 2014 Mar;9(3):626-32. Epub 2013 Aug 29 doi: 10.2215/CJN.05810513. PMID: 23990165Free PMC Article

Therapy

Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective.
Shabaka A, Tato Ribera A, Fernández-Juárez G
Nephron 2020;144(9):413-427. Epub 2020 Jul 28 doi: 10.1159/000508099. PMID: 32721952
Endothelin: 30 Years From Discovery to Therapy.
Barton M, Yanagisawa M
Hypertension 2019 Dec;74(6):1232-1265. Epub 2019 Nov 4 doi: 10.1161/HYPERTENSIONAHA.119.12105. PMID: 31679425
Nephrotic Syndrome.
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Focal Segmental Glomerulosclerosis.
Rosenberg AZ, Kopp JB
Clin J Am Soc Nephrol 2017 Mar 7;12(3):502-517. Epub 2017 Feb 27 doi: 10.2215/CJN.05960616. PMID: 28242845Free PMC Article
Pathology of IgA nephropathy.
Roberts IS
Nat Rev Nephrol 2014 Aug;10(8):445-54. Epub 2014 May 27 doi: 10.1038/nrneph.2014.92. PMID: 24861083

Prognosis

Proteinuria Reduction and Kidney Survival in Focal Segmental Glomerulosclerosis.
Troost JP, Trachtman H, Spino C, Kaskel FJ, Friedman A, Moxey-Mims MM, Fine RN, Gassman JJ, Kopp JB, Walsh L, Wang R, Gipson DS
Am J Kidney Dis 2021 Feb;77(2):216-225. Epub 2020 Aug 10 doi: 10.1053/j.ajkd.2020.04.014. PMID: 32791086Free PMC Article
Quantification of cancer risk in glomerulonephritis.
Heaf JG, Hansen A, Laier GH
BMC Nephrol 2018 Feb 2;19(1):27. doi: 10.1186/s12882-018-0828-2. PMID: 29394927Free PMC Article
Molecular profiling in IgA nephropathy and focal and segmental glomerulosclerosis.
Tycová I, Hrubá P, Maixnerová D, Girmanová E, Mrázová P, Straňavová L, Zachoval R, Merta M, Slatinská J, Kollár M, Honsová E, Tesař V, Viklický O
Physiol Res 2018 Mar 16;67(1):93-105. Epub 2017 Nov 10 doi: 10.33549/physiolres.933670. PMID: 29137483
Obesity and chronic kidney disease.
Eknoyan G
Nefrologia 2011;31(4):397-403. Epub 2011 May 30 doi: 10.3265/Nefrologia.pre2011.May.10963. PMID: 21623393
The incidence of primary glomerulonephritis worldwide: a systematic review of the literature.
McGrogan A, Franssen CF, de Vries CS
Nephrol Dial Transplant 2011 Feb;26(2):414-30. Epub 2010 Nov 10 doi: 10.1093/ndt/gfq665. PMID: 21068142

Clinical prediction guides

Recurrent Glomerular Disease after Kidney Transplantation: Diagnostic and Management Dilemmas.
Uffing A, Hullekes F, Riella LV, Hogan JJ
Clin J Am Soc Nephrol 2021 Nov;16(11):1730-1742. Epub 2021 Oct 22 doi: 10.2215/CJN.00280121. PMID: 34686531Free PMC Article
Kidney disease in the setting of HIV infection: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.
Swanepoel CR, Atta MG, D'Agati VD, Estrella MM, Fogo AB, Naicker S, Post FA, Wearne N, Winkler CA, Cheung M, Wheeler DC, Winkelmayer WC, Wyatt CM; Conference Participants
Kidney Int 2018 Mar;93(3):545-559. Epub 2018 Feb 3 doi: 10.1016/j.kint.2017.11.007. PMID: 29398134Free PMC Article
Oxford Classification of IgA nephropathy 2016: an update from the IgA Nephropathy Classification Working Group.
Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, Liu ZH, Roberts IS, Yuzawa Y, Zhang H, Feehally J; IgAN Classification Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Conference Participants
Kidney Int 2017 May;91(5):1014-1021. Epub 2017 Mar 22 doi: 10.1016/j.kint.2017.02.003. PMID: 28341274
Pathology of IgA nephropathy.
Roberts IS
Nat Rev Nephrol 2014 Aug;10(8):445-54. Epub 2014 May 27 doi: 10.1038/nrneph.2014.92. PMID: 24861083
Glomerular diseases: FSGS.
Bose B, Cattran D; Toronto Glomerulonephritis Registry
Clin J Am Soc Nephrol 2014 Mar;9(3):626-32. Epub 2013 Aug 29 doi: 10.2215/CJN.05810513. PMID: 23990165Free PMC Article

Recent systematic reviews

Interventions for focal segmental glomerulosclerosis in adults.
Hodson EM, Sinha A, Cooper TE
Cochrane Database Syst Rev 2022 Feb 28;2(2):CD003233. doi: 10.1002/14651858.CD003233.pub3. PMID: 35224732Free PMC Article
Rituximab therapy for focal segmental glomerulosclerosis and minimal change disease in adults: a systematic review and meta-analysis.
Hansrivijit P, Cheungpasitporn W, Thongprayoon C, Ghahramani N
BMC Nephrol 2020 Apr 15;21(1):134. doi: 10.1186/s12882-020-01797-7. PMID: 32293308Free PMC Article
Rituximab treatment for relapsing minimal change disease and focal segmental glomerulosclerosis: a systematic review.
Kronbichler A, Kerschbaum J, Fernandez-Fresnedo G, Hoxha E, Kurschat CE, Busch M, Bruchfeld A, Mayer G, Rudnicki M
Am J Nephrol 2014;39(4):322-30. Epub 2014 Apr 15 doi: 10.1159/000360908. PMID: 24751753
The incidence of primary glomerulonephritis worldwide: a systematic review of the literature.
McGrogan A, Franssen CF, de Vries CS
Nephrol Dial Transplant 2011 Feb;26(2):414-30. Epub 2010 Nov 10 doi: 10.1093/ndt/gfq665. PMID: 21068142
Immunosuppressive treatment for focal segmental glomerulosclerosis in adults.
Braun N, Schmutzler F, Lange C, Perna A, Remuzzi G, Risler T, Willis NS
Cochrane Database Syst Rev 2008 Jul 16;2008(3):CD003233. doi: 10.1002/14651858.CD003233.pub2. PMID: 18646090Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...