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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

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
Approach to Diagnosis and Management of Primary Glomerular Diseases Due to Podocytopathies in Adults: Core Curriculum 2020.
Ahn W, Bomback AS
Am J Kidney Dis 2020 Jun;75(6):955-964. Epub 2020 Apr 21 doi: 10.1053/j.ajkd.2019.12.019. PMID: 32331832
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
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
Focal segmental glomerulosclerosis.
D'Agati VD, Kaskel FJ, Falk RJ
N Engl J Med 2011 Dec 22;365(25):2398-411. doi: 10.1056/NEJMra1106556. PMID: 22187987

Prognosis

Incidence and risk factors for recurrent focal segmental glomerulosclerosis after kidney transplantation: a meta-analysis.
Bai J, Zhang T, Wang Y, Cao J, Duan Z, Ji L, Zhou Y, Hao C, Guo Q
Ren Fail 2023 Dec;45(1):2201341. doi: 10.1080/0886022X.2023.2201341. PMID: 37070350Free PMC Article
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
Genetics of childhood steroid-sensitive nephrotic syndrome.
Karp AM, Gbadegesin RA
Pediatr Nephrol 2017 Sep;32(9):1481-1488. Epub 2016 Jul 29 doi: 10.1007/s00467-016-3456-8. PMID: 27470160Free PMC Article
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
Collapsing glomerulopathy.
Schwimmer JA, Markowitz GS, Valeri A, Appel GB
Semin Nephrol 2003 Mar;23(2):209-18. doi: 10.1053/snep.2003.50019. PMID: 12704581

Clinical prediction guides

Rituximab treatment for refractory nephrotic syndrome in adults: a multicenter retrospective study.
Ma X, Fang L, Sheng L, Zhou X, Bai S, Zang X, Wang Y, Li M, Lv Z, Zhong Q, Yang X, Wang Y, Hu Y, Yan D, Shi Y, Chen H, Li J, Tao M, Zhuang S, Wang Y, Liu N
Ren Fail 2023 Dec;45(1):2237124. doi: 10.1080/0886022X.2023.2237124. PMID: 37482915Free 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

Prevalence and distribution of primary glomerular diseases in Africa: a systematic review and meta-analysis of observational studies.
Ekrikpo UE, Obiagwu PN, Udo AI, Chukwuonye II, Noubiap JJ, Okpechi-Samuel US, Udoudo UN, Tannor EK, Ngoka SC, Mbah IO, Bello AK, Okpechi IG
Pan Afr Med J 2023;45:153. Epub 2023 Aug 9 doi: 10.11604/pamj.2023.45.153.40741. PMID: 37869232Free PMC Article
Incidence, risk factors, and outcomes of recurrent focal segmental glomerulosclerosis in pediatric kidney transplant recipients: A systematic review and meta-analysis.
Bai J, Yin X, Li J, Li JQ, Niu Y, Li Z, Li J, Zhou Y
Clin Transplant 2023 Nov;37(11):e15119. Epub 2023 Sep 19 doi: 10.1111/ctr.15119. PMID: 37725070
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
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

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