MyEClinic
MyEClinic
Health Dictionary Find a Doctor

Focal segmental glomerulosclerosis

Template:DiseaseDisorder infobox

For patient information on this page, click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: ; M. Khurram Afzal, MD [3]; Manpreet Kaur, MD [4]; Olufunmilola Olubukola M.D.[5]; Cafer Zorkun, M.D., Ph.D. [6]; Syed Hassan A. Kazmi BSc, MD [7]; Synonyms and keywords: FSGS

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]; Associate Editor-In-Chief:’’’Ayesha A. Khan, MD[3] Olufunmilola Olubukola M.D.[4] Cafer Zorkun, M.D., Ph.D. [5]

Overview

Focal segmental glomerulosclerosis (FSGS) is a cause of nephrotic syndrome in children and adolescents, as well as an important cause of kidney failure in adults.[1] Focal Segmental Glomerulosclerosis (FSGS) is a distinct finding and a descriptive report on the glomerular morphology seen in certain glomerular disease conditions. It is considered to be a glomerular podocytopathy [2]. Minimal change disease (MCD) is by far the most common cause of nephrotic syndrome in children: MCD and primary FSGS may have a similar cause.[1] Focal Segmental Glomerulosclerosis (FSGS) is very similar in presentation to Minimal Change Kidney Disease (MCD), in-fact both are podocytopathies but they are histological different with varying disease signs and symptoms.

The individual components of the name refer to the appearance of the kidney tissue on biopsy: focal – only some of the glomeruli are involved (as opposed to diffuse), segmental – only part of an entire glomerulus is involved (as opposed to global), glomerulosclerosis – refers to scarring of the glomerulus (a part of the nephron (the functional unit of the kidney)).

FSGS is a progressive form of renal disease, it has become the most common cause of GN-related ESRD in patients with End Stage Renal Disease (ESRD) in the United States. [3] It accounts for about 40% of adults and about 20% of pediatric cases of Nephrotic Syndrome in the United States. [3]

FSGS can be a primary or secondary cause of Nephrotic Syndrome. Primary FSGS can occur as an epithelial cell disorder characterized by podocytopathy of the glomeruli that may be related etiologically to minimal change disease. Secondary FSGS occurs due to previous glomerular injury or as a response to previous nephron loss from toxic effects of drugs, viral infections and chronic systemic diseases like diabetes mellitus, SLE, and other renal affecting autoimmune diseases.

Definitive diagnosis of FSGS is by kidney biopsy. High clinical suspicion is also very important in the diagnosis and differentiation of FSGS from MCD. MCD often presents with features of Nephrotic Syndrome like proteinuria, edema and hyperlipidemia while FSGS may present with hematuria, hypertension and decreased renal function which are common presentations of nephritic syndrome.

Historical Perspective

Focal segmental glomerulosclerosis (FSGS) was first discovered by a Theodor Fahr, a German pathologist, in 1925, and he referred to it as “lipoid nephrosis with degeneration“, showing a clear association to minimal change disease. In 1957, FSGS was then described by Dr. Arnold Rich, a pathologist at Johns Hopkins University.

Classification

FSGS can be classified as primary and secondary disease depending on etiology, the course of the disease and histologic pattern.

Pathophysiology

The pathophysiology of focal segmental glomerulosclerosis (FSGS) is based on two types of FSGS. Primary FSGS is also known as idiopathic FSGS, there is a hypothesis that suggests it occurs as a result of circulating immune activating factors interacting with the glomerular epithelium. The underlying pathogenesis of FSGS is fusion or effacement of the foot processes (podocytes) of the glomeruli and sclerosing of some parts of the glomeruli. These changes result in apoptosis and detachment of the glomerular basement membrane (GBM) resulting in subsequent loss of negative charge on podocytes and podocytopenia. Secondary FSGS is based on glomerular hypertrophy and hyperfiltration and over expression of inflammatory mediators such as, TGF-beta, PDGF and VEGF. The underlying pathogenesis can be based on multiple genetic mutations in NPHS1, NEPH1, NPHS2, WT1 and INF2 genes. Conditions associated with FSGS include, diabetes, HIV, sickle cell disease, nephrotic syndrome and minimal change disease. On microscopic histopathological analysis progressive changes seen are, foot process effacement, podocyte apoptosis, exposed GBM, capillary expansion and mesangial matrix proliferation.

Causes

Differentials

Risk Factors

Screening

Epidemiology and Demographics

Natural History, Complications, and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

  1. ↑ 1.0 1.1 Kumar V, Fausto N, Abbas A (editors) (2003). Robbins & Cotran Pathologic Basis of Disease (7th ed.). Saunders. pp. pp. 982-3. ISBN 978-0-721-60187-8.
  2. ↑ Sethi S, Glassock RJ, Fervenza FC (2015). “Focal segmental glomerulosclerosis: towards a better understanding for the practicing nephrologist”. Nephrol Dial Transplant. 30 (3): 375–84. doi:10.1093/ndt/gfu035. PMID 24589721 PMID: 24589721 Check |pmid= value (help).
  3. ↑ 3.0 3.1 Kitiyakara C, Eggers P, Kopp JB (2004). “Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States”. Am J Kidney Dis. 44 (5): 815–25. PMID 15492947.


Template:WH Template:WS

Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [3], Manpreet Kaur, MD [4], Olufunmilola Olubukola M.D.[5]

Overview

Focal segmental glomerulosclerosis (FSGS) was discovered by Theodor Fahr, a German pathologist, in 1925, and he referred to it as “lipoid nephrosis with degeneration“, showing a clear association to minimal change disease. In 1957, FSGS was then described by Dr. Arnold Rich, a pathologist at Johns Hopkins University.

Historical Perspective

Discovery


References

  1. ↑ Fahr, T (1925). Pathologische anatomie des morbus brightii. In: Fahr T, Gruber GB, Koch M, et al. eds. Harnorgane Männliche Geschlechtsorgane. Vienna: Springer. pp. 156–472.
  2. ↑ Fahr, T (1925). Pathologische anatomie des morbus brightii. In: Fahr T, Gruber GB, Koch M, et al. eds. Harnorgane Männliche Geschlechtsorgane. Vienna: Springer. pp. 156–472.
  3. ↑ RICH AR (1957). “A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis”. Bull Johns Hopkins Hosp. 100 (4): 173–86. PMID 13426687.
  4. ↑ Churg J, Habib R, White RH (1970). “Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children”. Lancet. 760 (1): 1299–302. PMID 4193942.

Template:WH Template:WS

Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2], Manpreet Kaur, MD [3]

Overview

FSGS can be classified as primary and secondary disease depending on etiology, the course of the disease and histologic pattern.

Classification

FSGS can be classified as primary and secondary disease depending on etiology, the course of the disease and histologic pattern:

The Columbia classification of focal segmental glomerulosclerosis (FSGS) based on morphology by D’Agati[2]

Pathological Classification of Focal Segmental Glomerulosclerosis
Variant Location Distribution Features
Not Otherwise Specified (NOS) Anywhere Segmental Capillary lumen abolished by the segmental increase in the matrix.
Perihilar Variant Perihilar Segmental Presence of one or more glomeruli containing hyalinosis in the perihilar regions with or without sclerosis. Within each glomerulus, the segmental lesions must contain > 50% perihilar hyalinosis and/or sclerosis.
Cellular Variant Anywhere Segmental Presence of one or more glomerulus with segmental hypercellularity of the capillary endothelium that blocks the capillary lumen, with or without foam cells and/or karyorrhexis.
Tip Variant At tip domain Segmental One or more segmental lesions, that include tip domains. Lesions must have adhesions/confluence of podocytes with parietal or tubular cells. Tip domains are defined as 25% of tuft adjacent to the origin of the proximal tubule. Sclerosing lesions shuld be <25% of tuft, while cellular lesions should be < 50% of tuft. No perihilar sclerosis should be observed.
Collapsing Variant Anywhere Segmental or global One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia.
Adapted from D’Agati VD, Fogo AB, Bruijn JA, and Jennette JC. Pathological classification of focal segmental glomerulosclerosis. A working proposal. Am J of Kidney Dis. 2004; 43(2):368-382.

References

  1. ↑ Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL; et al. (2001). “Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1”. J Am Soc Nephrol. 12 (7): 1434–47. PMID 11423572 PMID 11423572 Check |pmid= value (help).
  2. ↑ D’Agati VD, Fogo AB, Bruijn JA, Jennette JC (2004). “Pathologic classification of focal segmental glomerulosclerosis: a working proposal”. Am J Kidney Dis. 43 (2): 368–82. PMID 14750104.


Template:WH Template:WS

Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [3], Manpreet Kaur, MD [4], Cafer Zorkun, M.D., Ph.D. [5], Olufunmilola Olubukola M.D.[6]

Overview

The pathophysiology of focal segmental glomerulosclerosis (FSGS) is based on two types of FSGS. Primary FSGS is also known as idiopathic FSGS, there is a hypothesis that suggests it occurs as a result of circulating immune activating factors interacting with the glomerular epithelium. The underlying pathogenesis of FSGS is fusion or effacement of the foot processes (podocytes) of the glomeruli and sclerosing of some parts of the glomeruli. These changes result in apoptosis and detachment of the glomerular basement membrane (GBM) resulting in subsequent loss of negative charge on podocytes and podocytopenia. Secondary FSGS is based on glomerular hypertrophy and hyperfiltration and over expression of inflammatory mediators such as, TGF-beta, PDGF and VEGF. The underlying pathogenesis can be based on multiple genetic mutations in NPHS1, NEPH1, NPHS2, WT1 and INF2 genes. Conditions associated with FSGS include, diabetes, HIV, sickle cell disease, nephrotic syndrome and minimal change disease. On microscopic histopathological analysis progressive changes seen are, foot process effacement, podocyte apoptosis, exposed GBM, capillary expansion and mesangial matrix proliferation.

Pathophysiology

There are two types of FSGS, primary FSGS and secondary FSGS, pathophysiology is discussed below:

Pathogenesis of primary FSGS

Pathogenesis of secondary FSGS

The pathogenesis of secondary focal segmental glomerulosclerosis (FSGS) occurs due to the following factors:

Genetics

The development of focal segmental glomerulosclerosis is the result of multiple genetic mutations such as:[12][10][13][14][15][16][17][18]

Associated Conditions

Conditions associated with focal segmental glomerulosclerosis (FSGS):[19][20][21][22][23][24][25]

Microscopic Pathology

On microscopic histopathological analysis progressive changes seen are:[10][26]

References

  1. ↑ 1.0 1.1 1.2 1.3 Reiser J, Nast CC, Alachkar N (2014). “Permeability factors in focal and segmental glomerulosclerosis”. Adv Chronic Kidney Dis. 21 (5): 417–21. doi:10.1053/j.ackd.2014.05.010. PMC 4149759. PMID 25168830 PMID 25168830 Check |pmid= value (help).
  2. ↑ Asanuma K, Mundel P (2003). “The role of podocytes in glomerular pathobiology”. Clin Exp Nephrol. 7 (4): 255–9. doi:10.1007/s10157-003-0259-6. PMID 14712353.
  3. ↑ 3.0 3.1 Fogo AB (2003). “Animal models of FSGS: lessons for pathogenesis and treatment”. Semin Nephrol. 23 (2): 161–71. doi:10.1053/snep.2003.50015. PMID 12704576.
  4. ↑ 4.0 4.1 Wei C, Trachtman H, Li J, Dong C, Friedman AL, Gassman JJ; et al. (2012). “Circulating suPAR in two cohorts of primary FSGS”. J Am Soc Nephrol. 23 (12): 2051–9. doi:10.1681/ASN.2012030302. PMC 3507361. PMID 23138488.
  5. ↑ Rea R, Smith C, Sandhu K, Kwan J, Tomson C (2001). “Successful transplant of a kidney with focal segmental glomerulosclerosis”. Nephrol Dial Transplant. 16 (2): 416–7. PMID 11158426.
  6. ↑ Ghiggeri GM, Artero M, Carraro M, Perfumo F (2001). “Permeability plasma factors in nephrotic syndrome: more than one factor, more than one inhibitor”. Nephrol Dial Transplant. 16 (5): 882–5. PMID 11328888.
  7. ↑ Kemper MJ, Wolf G, Müller-Wiefel DE (2001). “Transmission of glomerular permeability factor from a mother to her child”. N Engl J Med. 344 (5): 386–7. doi:10.1056/NEJM200102013440517. PMID 11195803.
  8. ↑ Harris RC, Neilson EG (2006). “Toward a unified theory of renal progression”. Annu Rev Med. 57: 365–80. doi:10.1146/annurev.med.57.121304.131342. PMID 16409155.
  9. ↑ Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL; et al. (2001). “Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1”. J Am Soc Nephrol. 12 (7): 1434–47. PMID 11423572.
  10. ↑ 10.0 10.1 10.2 Kwoh C, Shannon MB, Miner JH, Shaw A (2006). “Pathogenesis of nonimmune glomerulopathies”. Annu Rev Pathol. 1: 349–74. doi:10.1146/annurev.pathol.1.110304.100119. PMID 18039119.
  11. ↑ Hostetter TH (2003). “Hyperfiltration and glomerulosclerosis”. Semin Nephrol. 23 (2): 194–9. doi:10.1053/anep.2003.50017. PMID 12704579.
  12. ↑ Kestilä M, Lenkkeri U, Männikkö M, Lamerdin J, McCready P, Putaala H; et al. (1998). “Positionally cloned gene for a novel glomerular protein–nephrin–is mutated in congenital nephrotic syndrome”. Mol Cell. 1 (4): 575–82. PMID 9660941.
  13. ↑ Tryggvason K, Patrakka J, Wartiovaara J (2006). “Hereditary proteinuria syndromes and mechanisms of proteinuria”. N Engl J Med. 354 (13): 1387–401. doi:10.1056/NEJMra052131. PMID 16571882.
  14. ↑ Kim JM, Wu H, Green G, Winkler CA, Kopp JB, Miner JH; et al. (2003). “CD2-associated protein haploinsufficiency is linked to glomerular disease susceptibility”. Science. 300 (5623): 1298–300. doi:10.1126/science.1081068. PMID 12764198.
  15. ↑ Shih NY, Li J, Karpitskii V, Nguyen A, Dustin ML, Kanagawa O; et al. (1999). “Congenital nephrotic syndrome in mice lacking CD2-associated protein”. Science. 286 (5438): 312–5. PMID 10514378.
  16. ↑ Kaplan JM, Kim SH, North KN, Rennke H, Correia LA, Tong HQ; et al. (2000). “Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis”. Nat Genet. 24 (3): 251–6. doi:10.1038/73456. PMID 10700177.
  17. ↑ Winn MP (2003). “Approach to the evaluation of heritable diseases and update on familial focal segmental glomerulosclerosis”. Nephrol Dial Transplant. 18 Suppl 6: vi14–20. PMID 12953036.
  18. ↑ Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH; et al. (2013). “KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis”. Am J Kidney Dis. 62 (3): 403–41. doi:10.1053/j.ajkd.2013.06.002. PMID 23871408.
  19. ↑ Hogan J, Radhakrishnan J (April 2013). “The treatment of minimal change disease in adults”. J. Am. Soc. Nephrol. 24 (5): 702–11. doi:10.1681/ASN.2012070734. PMID 23431071.
  20. ↑ Collins AJ, Foley RN, Herzog C, Chavers B, Gilbertson D, Ishani A, Kasiske B, Liu J, Mau LW, McBean M, Murray A, St Peter W, Guo H, Gustafson S, Li Q, Li S, Li S, Peng Y, Qiu Y, Roberts T, Skeans M, Snyder J, Solid C, Wang C, Weinhandl E, Zaun D, Arko C, Chen SC, Dalleska F, Daniels F, Dunning S, Ebben J, Frazier E, Hanzlik C, Johnson R, Sheets D, Wang X, Forrest B, Constantini E, Everson S, Eggers P, Agodoa L (January 2011). “US Renal Data System 2010 Annual Data Report”. Am. J. Kidney Dis. 57 (1 Suppl 1): A8, e1–526. doi:10.1053/j.ajkd.2010.10.007. PMID 21184928.
  21. ↑ Cohen AH, Nast CC (March 1988). “HIV-associated nephropathy. A unique combined glomerular, tubular, and interstitial lesion”. Mod. Pathol. 1 (2): 87–97. PMID 3070550.
  22. ↑ Ataga KI, Derebail VK, Archer DR (September 2014). “The glomerulopathy of sickle cell disease”. Am. J. Hematol. 89 (9): 907–14. doi:10.1002/ajh.23762. PMC 4320776. PMID 24840607.
  23. ↑ Gopalakrishnan I, Iskandar SS, Daeihagh P, Divers J, Langefeld CD, Bowden DW, Hicks PJ, Rocco MV, Freedman BI (February 2011). “Coincident idiopathic focal segmental glomerulosclerosis collapsing variant and diabetic nephropathy in an African American homozygous for MYH9 risk variants”. Hum. Pathol. 42 (2): 291–4. doi:10.1016/j.humpath.2010.07.016. PMC 3022108. PMID 21074826.
  24. ↑ Hanaoka H, Hashiguchi A, Konishi K, Kuwana M, Takeuchi T (May 2015). “An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?”. CEN Case Rep. 4 (1): 70–75. doi:10.1007/s13730-014-0142-1. PMC 5411626. PMID 28509272.
  25. ↑ Brown EJ, Pollak MR, Barua M (May 2014). “Genetic testing for nephrotic syndrome and FSGS in the era of next-generation sequencing”. Kidney Int. 85 (5): 1030–8. doi:10.1038/ki.2014.48. PMC 4118212. PMID 24599252.
  26. ↑ Reidy K, Kaskel FJ (March 2007). “Pathophysiology of focal segmental glomerulosclerosis”. Pediatr. Nephrol. 22 (3): 350–4. doi:10.1007/s00467-006-0357-2. PMC 1794138. PMID 17216262.


Template:WH Template:WS

Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

Common Causes

[Disease name] may be caused by:

  • [Cause1]
  • [Cause2]
  • [Cause3


  • [Disease name] is caused by an infection with [pathogen name].
  • [Pathogen name] is caused by [pathogen name].

Less Common Causes

Less common causes of disease name include:

  • [Cause1]
  • [Cause2]
  • [Cause3]

Genetic Causes

Virus Associated

Medication

=


Malignancy

References


Template:WH Template:WS

Differentiating Focal segmental glomerulosclerosis from other Diseases

Differentiating Focal segmental glomerulosclerosis from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Overview

Focal segmental glomerulosclerosis should be differentiate from other causes of glomerular disease such as nephritic syndrome, nephrotic syndrome, Fabry’s disease, poststreptococcal glomerulonephritis, lupus nephritis, antiglomerular basement membrane disease (goodpasture’s syndrome), Cryoglobulinemia, Henoch-Schönlein purpura, amyloidosis, pulmonary-renal syndromes (vasculitis), thin basement membrane disease, Alport’s Syndrome, anti-GBM Disease, hypertensive nephrosclerosis, and subacute bacterial endocarditis. The various types of glomerular diseases may be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features.

Differential Diagnosis

Focal segmental glomerulosclerosis should be differentiate from other causes of glomerular disease. The various types of glomerular diseases may be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features. The following table differentiates between various types of glumerular diseases:

Glomerular diseases Disease History and Symtoms Laboratory Findings Pathology
History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Acute Nephritic Syndromes Poststreptococcal Glomerulonephritis[1][2][3] +/- + +/- +/- +/- +/- +/- +/-
  • Immune complex GN
  • Granular deposit
Renal disease due to Subacute Bacterial Endocarditis, or cardiac shunt (Atrioventricular)[4][5] +/- + +/- +/- +/- +/- +/- +/-
  • Crescentic GN is the most common pathological features
  • Mesangial deposits,
  • Subendothelial deposits
  • Subepithelial “humps,” in minority of cases
  • Pauci-immune GN
Lupus Nephritis[6]
  • History of SLE features
 +/- + +/- +/- +/- +/- +/- +/-
  • Differs based on the disease classification
  • Differs based on the disease classification
  • Differs based on the disease classification, mostly immune complex GN
  • Granular deposit
Antiglomerular Basement Membrane Disease (Goodpasture’s syndrome)[7][8]
  • Young adults
 + + + + + + – – Diffuse thickening of the glomerular basement membrane with absence of sub-epithelial and sub-endothelial deposits 
  • Immune complex GN
  • Linear deposit
IgA Nephropathy[9][10] + +/- + +/- + – + –
  • Immune complex deposition
  • Crescent formation
  • Immune complex GN, granular deposite
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
ANCA Small-Vessel Vasculitis[11][12] Granulomatosis with Polyangiitis (Wegener’s)[13][14][15]
  • Middle age male
 + + + +/- + – + –
  •  Pauci-immune GN
Microscopic Polyangiitis[16] +/- + + + + + + –
  •  Pauci-immune GN
Churg-Strauss Syndrome[17] +/- + + + + + + –
  •  Pauci-immune GN
Membranoproliferative Glomerulonephritis[18][19] + + + +/- + + – – –
  • Immune complex GN
  • Granular deposite
Henoch-Schönlein purpura [20] + + + +/- + + – – –
  • Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
  • Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
  • Immune complex GN, granular deposite
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Cryoglobulinemia[21] Patients having cryoglobulinemia may have positive history of: Pulmonary symptoms:
  • Cough

Cutaneous symptoms:

Gastrointestinal symptoms:

  • Abdominal pain

General symptoms:

+/- + +/- + +/- +/- +/- +/- +/-
  • Prominent IgM and C3
Nephrotic Syndrome Minimal Change Disease[22][23] – + – + +/- + – +
  • Normal
 –
Focal Segmental Glomerulosclerosis[24][25][26] – + – + +/- + – + –
Membranous Glomerulonephritis[27][28] – + – + +/- + – + Immune complex deposition Immune complex GN, granular deposite
Diabetic Nephropathy[29][30][31][32][33][34][35][36][37][38] For more information on diabetes click here. – + – + +/- + – +
  • Diffuse mesangial matrix expansion (nodular glomerulosclerosis)
  • Increased mesangial hypercellularity
  • Prominent glomerular basement membranes
  • Thick basement membrane without any deposit
  • Nodular glomerulosclerosis
 –
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
 Glomerular Deposition Diseases  Light Chain Deposition Disease[39]
  • Occurs in the setting of high tumor burden
 – – + – + +/- + – + –
  • Light-chain deposits
  • Granular deposits on electron microscopy
  • Detection of light chain deposits using anti–light chain antibody
Renal Amyloidosis[40][41][42][43] – + – + +/- + – + –
  • Diffuse glomerular deposition of amorphous hyaline material (nodular pattern), in mesangium (weakly staining with periodic acid-Schiff (PAS)
  • Nodular deposit
  • AA amyloidosis type: negative for immunoglobulins and complement
  • AL amyloidosis type: Positive for lambda or kappa light chains
Fibrillary-Immunotactoid Glomerulopathy[44] – +/- + +/- +/- +/- + +/- +/- –
  • Diffuse sclerosing glomerulonephritis
  • Diffuse proliferative glomerulonephritis
  • Membranoproliferative glomerulonephritis
  • Mesangioproliferative/sclerosing disease
  • Membranous glomerulonephritis
  • Large fibrillar deposits in the mesangium randomly
  • Glomerular capillary walls different from amloidosis
  • No staining with Congo red or thioflavine-T or with antibodies to a specific type
  • Positive for immunoglobulin G (IgG), C3
  • Kappa and lambda (ie, polyclonal) light chains
Fabry’s Disease[45][46][47] – + – + +/- + – + –
  • Vacuolization of visceral glomerular epithelial cells (podocytes) and distal tubular epithelial cells
  • Glycolipid accumulation
  • Myeloid or zebra bodies: Gb3 deposition within enlarged secondary lysosomes as lamellated membrane structures
  • Inclusions, composed of concentric layers (onion skin appearance)
 –
Basement Membrane Syndrome Alport’s Syndrome[48][49][50][51][52][53]
  • Positive family history
 Auditary:

Occular problems:

  • Refractory Error
 – + – + +/- + – + –
  • Early stage: unremarkable
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Thin Basement Membrane Disease[54][55]
  • Positive family history
 – – + -/+ – -/+ – -/+ – – – Diffuse thinning of the glomerular basement membranes (GBM) –
Nail-Patella Syndrome[56][57]
  • Positive family history
  • Poorly developed fingernails, toe nails, and patellae (kneecaps).
  • Elbow deformities
  • Abnormally shaped pelvis bone (hip bone)
  • Knee may be small, deformed or absent
 + + – – – – – – –
  • Mostly unremarkable changes
  • Secondary FSGS
  • Late stages:
    • Global glomerulosclerosis,
    • Tubulointerstitial fibrosis
  • Glomerular basement membranes (GBMs): Focal or diffuse irregular thickening with electron-lucent areas (moth-eaten appearance) containing type III collagen bundles.
  • Similar collagen fibrils can be seen in mesangial matrix.
  • Podocytes: Segmental effacement of foot processes.
  • Nonspecific IgM and C3 deposition may be seen in sclerotic glomeruli.
 Glomerular-Vascular Syndromes  Hypertensive Nephrosclerosis[58] Chronic hypertension +/- +/- + +/- +/- +/- – +/- –
  • Interstitial fibrosis and atrophy
  • Medial thickening and intimal fibrosis of medium-sized and larger vessels
  • Arteriolar thickening, and hyalinosis
  • Chronic stages:
Cholesterol Emboli[59]
  • Depends on the organ involved
 +/- +/- + +/- +/- +/- – +/- –
  • Atheroemboli are seen in interlobular and arcuate arteries, as lance-shaped clefts, due to dissolution of cholesterol crystals
  • Acute lesions:
    • Atheroemboli are surrounded by red blood cells, fibrin, and leukocytes, with multinucleated giant cell reactions
  • Chronic lesions:
    • Cholesterol clefts are surrounded by intimal fibrosis
    • Vessel recanalization of chronic lesions can occur.
  • Global and segmental sclerosis of glomeruli may be present.
  • Extensive foot process effacement can be seen
  • Not specific changes
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Sickle Cell Disease[60]
  • Positive family history
 +/- +/- +/- – – – – – –
  • Glomerular hypertrophy
  • Hemosiderin deposits
  • Focal areas of hemorrhage or necrosis
  • Chronic stage: interstitial inflammation, edema, fibrosis, tubular atrophy, and papillary infarcts
  • Glomerular enlargement and focal segmental glomerulosclerosis (FSGS)
Thrombotic Microangiopathies[61] Click for more information on Thrombotic Microangiopathies. + +/- + +/- +/- +/- – – –
  • Acute stage:
    • Inravasculr fibrin thrombi
  • Chronic stage:
    • Endocapillary hypercellularity.
    • Intimal proliferation of arterioles
  • Swollen glomerular endothelial cells with loss of fenestrations
  • Chronic stage: interposed cells with new GBM matrix material deposition.
Antiphospholipid Antibody Syndrome [62][63][64]
  • Fatigue
  • Fever
  • Weight loss
 + +/- + +/- +/- +/- – – –
  • Swollen glomerular endothelial cells with loss of fenestrations
  • Chronic stage: interposed cells with new GBM matrix material deposition.


Some infectious diseases such as HIV, HBV, HCV, syphilis, leprosy, malaria, and schistosomiasis may cause glomerular diseases.

References

  1. ↑ GERMUTH FG (1953). “A comparative histologic and immunologic study in rabbits of induced hypersensitivity of the serum sickness type”. J Exp Med. 97 (2): 257–82. PMC 2136196. PMID 13022878.
  2. ↑ Germuth FG, Senterfit LB, Dreesman GR (1972). “Immune complex disease. V. The nature of the circulating complexes associated with glomerular alterations in the chronic BSA-rabbit system”. Johns Hopkins Med J. 130 (6): 344–57. PMID 5031005.
  3. ↑ Radhakrishnan J, Cattran DC (2012). “The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines–application to the individual patient”. Kidney Int. 82 (8): 840–56. doi:10.1038/ki.2012.280. PMID 22895519.
  4. ↑ Neugarten J, Baldwin DS (August 1984). “Glomerulonephritis in bacterial endocarditis”. Am. J. Med. 77 (2): 297–304. PMID 6380288.
  5. ↑ Arze RS, Rashid H, Morley R, Ward MK, Kerr DN (January 1983). “Shunt nephritis: report of two cases and review of the literature”. Clin. Nephrol. 19 (1): 48–53. PMID 6831779.
  6. ↑ Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M (February 2004). “The classification of glomerulonephritis in systemic lupus erythematosus revisited”. Kidney Int. 65 (2): 521–30. doi:10.1111/j.1523-1755.2004.00443.x. PMID 14717922.
  7. ↑ Bolton WK (November 1996). “Goodpasture’s syndrome”. Kidney Int. 50 (5): 1753–66. PMID 8914046.
  8. ↑ Mathew TH, Hobbs JB, Kalowski S, Sutherland PW, Kincaid-Smith P (February 1975). “Goodpasture’s syndrome: normal renal diagnostic findings”. Ann. Intern. Med. 82 (2): 215–8. PMID 1090223.
  9. ↑ Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA (October 2011). “The pathophysiology of IgA nephropathy”. J. Am. Soc. Nephrol. 22 (10): 1795–803. doi:10.1681/ASN.2011050464. PMC 3892742. PMID 21949093.
  10. ↑ Wyatt RJ, Julian BA (June 2013). “IgA nephropathy”. N. Engl. J. Med. 368 (25): 2402–14. doi:10.1056/NEJMra1206793. PMID 23782179.
  11. ↑ Higgins RM, Goldsmith DJ, Connolly J, Scoble JE, Hendry BM, Ackrill P, Venning MC (January 1996). “Vasculitis and rapidly progressive glomerulonephritis in the elderly”. Postgrad Med J. 72 (843): 41–4. PMC 2398323. PMID 8746284.
  12. ↑ Jennette JC (March 2003). “Rapidly progressive crescentic glomerulonephritis”. Kidney Int. 63 (3): 1164–77. doi:10.1046/j.1523-1755.2003.00843.x. PMID 12631105.
  13. ↑ Renaudineau Y, Le Meur Y (October 2008). “Renal involvement in Wegener’s granulomatosis”. Clin Rev Allergy Immunol. 35 (1–2): 22–9. doi:10.1007/s12016-007-8066-6. PMID 18172777.
  14. ↑ Weiss MA, Crissman JD (October 1984). “Renal biopsy findings in Wegener’s granulomatosis: segmental necrotizing glomerulonephritis with glomerular thrombosis”. Hum. Pathol. 15 (10): 943–56. PMID 6384024.
  15. ↑ Pagnoux C (March 2008). “[Wegener’s granulomatosis and microscopic polyangiitis]”. Rev Prat (in French). 58 (5): 522–32. PMID 18524109.
  16. ↑ Chung SA, Seo P (August 2010). “Microscopic polyangiitis”. Rheum. Dis. Clin. North Am. 36 (3): 545–58. doi:10.1016/j.rdc.2010.04.003. PMC 2917831. PMID 20688249.
  17. ↑ Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C (May 2006). “Renal involvement in Churg-Strauss syndrome”. Am. J. Kidney Dis. 47 (5): 770–9. doi:10.1053/j.ajkd.2006.01.026. PMID 16632015.
  18. ↑ Alchi B, Jayne D (August 2010). “Membranoproliferative glomerulonephritis”. Pediatr. Nephrol. 25 (8): 1409–18. doi:10.1007/s00467-009-1322-7. PMC 2887509. PMID 19908070.
  19. ↑ Davis AE, Schneeberger EE, Grupe WE, McCluskey RT (May 1978). “Membranoproliferative glomerulonephritis (MPGN type I) and dense deposit disease (DDD) in children”. Clin. Nephrol. 9 (5): 184–93. PMID 657595.
  20. ↑ Jennette JC, Falk RJ (July 1994). “The pathology of vasculitis involving the kidney”. Am. J. Kidney Dis. 24 (1): 130–41. PMID 8023818.
  21. ↑ Fogo AB, Lusco MA, Najafian B, Alpers CE (February 2016). “AJKD Atlas of Renal Pathology: Cryoglobulinemic Glomerulonephritis”. Am. J. Kidney Dis. 67 (2): e5–7. doi:10.1053/j.ajkd.2015.12.007. PMID 26802335.
  22. ↑ Saha TC, Singh H (November 2006). “Minimal change disease: a review”. South. Med. J. 99 (11): 1264–70. doi:10.1097/01.smj.0000243183.87381.c2. PMID 17195422.
  23. ↑ Saleem MA, Kobayashi Y (2016). “Cell biology and genetics of minimal change disease”. F1000Res. 5. doi:10.12688/f1000research.7300.1. PMC 4821284. PMID 27092244.
  24. ↑ Rosenberg AZ, Kopp JB (March 2017). “Focal Segmental Glomerulosclerosis”. Clin J Am Soc Nephrol. 12 (3): 502–517. doi:10.2215/CJN.05960616. PMC 5338705. PMID 28242845.
  25. ↑ Jefferson JA, Shankland SJ (September 2014). “The pathogenesis of focal segmental glomerulosclerosis”. Adv Chronic Kidney Dis. 21 (5): 408–16. doi:10.1053/j.ackd.2014.05.009. PMC 4149756. PMID 25168829.
  26. ↑ Gephardt GN, Tubbs RR, Popowniak KL, McMahon JT (October 1986). “Focal and segmental glomerulosclerosis. Immunohistologic study of 20 renal biopsy specimens”. Arch. Pathol. Lab. Med. 110 (10): 902–5. PMID 2429634.
  27. ↑ Lai WL, Yeh TH, Chen PM, Chan CK, Chiang WC, Chen YM, Wu KD, Tsai TJ (February 2015). “Membranous nephropathy: a review on the pathogenesis, diagnosis, and treatment”. J. Formos. Med. Assoc. 114 (2): 102–11. doi:10.1016/j.jfma.2014.11.002. PMID 25558821.
  28. ↑ Wasserstein AG (April 1997). “Membranous glomerulonephritis”. J. Am. Soc. Nephrol. 8 (4): 664–74. PMID 10495797.
  29. ↑ Drummond K, Mauer M, International Diabetic Nephropathy Study Group (2002). “The early natural history of nephropathy in type 1 diabetes: II. Early renal structural changes in type 1 diabetes”. Diabetes. 51 (5): 1580–7. PMID 11978659.
  30. ↑ Hørlyck A, Gundersen HJ, Osterby R (1986). “The cortical distribution pattern of diabetic glomerulopathy”. Diabetologia. 29 (3): 146–50. PMID 3699305.
  31. ↑ Alpers CE, Hudkins KL (2011). “Mouse models of diabetic nephropathy”. Curr Opin Nephrol Hypertens. 20 (3): 278–84. doi:10.1097/MNH.0b013e3283451901. PMC 3658822. PMID 21422926.
  32. ↑ Kimmelstiel P, Wilson C (1936). “Intercapillary Lesions in the Glomeruli of the Kidney”. Am J Pathol. 12 (1): 83–98.7. PMC 1911022. PMID 19970254.
  33. ↑ Alpers CE, Biava CG (1989). “Idiopathic lobular glomerulonephritis (nodular mesangial sclerosis): a distinct diagnostic entity”. Clin Nephrol. 32 (2): 68–74. PMID 2766585.
  34. ↑ Toyoda M, Najafian B, Kim Y, Caramori ML, Mauer M (2007). “Podocyte detachment and reduced glomerular capillary endothelial fenestration in human type 1 diabetic nephropathy”. Diabetes. 56 (8): 2155–60. doi:10.2337/db07-0019. PMID 17536064.
  35. ↑ Najafian B, Crosson JT, Kim Y, Mauer M (2006). “Glomerulotubular junction abnormalities are associated with proteinuria in type 1 diabetes”. J Am Soc Nephrol. 17 (4 Suppl 2): S53–60. doi:10.1681/ASN.2005121342. PMID 16565248.
  36. ↑ Najafian B, Kim Y, Crosson JT, Mauer M (2003). “Atubular glomeruli and glomerulotubular junction abnormalities in diabetic nephropathy”. J Am Soc Nephrol. 14 (4): 908–17. PMID 12660325.
  37. ↑ Najafian B, Alpers CE, Fogo AB (2011). “Pathology of human diabetic nephropathy”. Contrib Nephrol. 170: 36–47. doi:10.1159/000324942. PMID 21659756.
  38. ↑ Najafian B, Alpers CE, Fogo AB (2011). “Pathology of human diabetic nephropathy”. Contrib Nephrol. 170: 36–47. doi:10.1159/000324942. PMID 21659756.
  39. ↑ Hutchison CA, Cockwell P, Stringer S, Bradwell A, Cook M, Gertz MA, Dispenzieri A, Winters JL, Kumar S, Rajkumar SV, Kyle RA, Leung N (June 2011). “Early reduction of serum-free light chains associates with renal recovery in myeloma kidney”. J. Am. Soc. Nephrol. 22 (6): 1129–36. doi:10.1681/ASN.2010080857. PMC 3103732. PMID 21511832.
  40. ↑ Baker KR, Rice L (2012). “The amyloidoses: clinical features, diagnosis and treatment”. Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
  41. ↑ Gillmore JD, Hawkins PN (October 2013). “Pathophysiology and treatment of systemic amyloidosis”. Nat Rev Nephrol. 9 (10): 574–86. doi:10.1038/nrneph.2013.171. PMID 23979488.
  42. ↑ Jerzykowska S, Cymerys M, Gil LA, Balcerzak A, Pupek-Musialik D, Komarnicki MA (2014). “Primary systemic amyloidosis as a real diagnostic challenge – case study”. Cent Eur J Immunol. 39 (1): 61–6. doi:10.5114/ceji.2014.42126. PMC 4439975. PMID 26155101.
  43. ↑ Pepys MB (2006). “Amyloidosis”. Annu. Rev. Med. 57: 223–41. doi:10.1146/annurev.med.57.121304.131243. PMID 16409147.
  44. ↑ Korbet SM, Schwartz MM, Lewis EJ (March 1991). “Immunotactoid glomerulopathy”. Am. J. Kidney Dis. 17 (3): 247–57. PMID 1996564.
  45. ↑ Alroy J, Sabnis S, Kopp JB (June 2002). “Renal pathology in Fabry disease”. J. Am. Soc. Nephrol. 13 Suppl 2: S134–8. PMID 12068025.
  46. ↑ Meikle PJ, Hopwood JJ, Clague AE, Carey WF (1999). “Prevalence of lysosomal storage disorders”. JAMA : the Journal of the American Medical Association. 281 (3): 249–54. PMID 9918480. Unknown parameter |month= ignored (help)
  47. ↑ Branton MH, Schiffmann R, Sabnis SG; et al. (2002). “Natural history of Fabry renal disease: influence of alpha-galactosidase A activity and genetic mutations on clinical course”. Medicine. 81 (2): 122–38. PMID 11889412. Unknown parameter |month= ignored (help)
  48. ↑ McCarthy PA, Maino DM (2000). “Alport syndrome: a review”. Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
  49. ↑ Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN; et al. (1993). “Hereditary nephritis (Alport’s syndrome)–clinical profile and inheritance in 28 kindreds”. Nephrol Dial Transplant. 8 (8): 690–5. PMID 8414153.
  50. ↑ Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN; et al. (1993). “Hereditary nephritis (Alport’s syndrome)–clinical profile and inheritance in 28 kindreds”. Nephrol Dial Transplant. 8 (8): 690–5. PMID 8414153.
  51. ↑ McCarthy PA, Maino DM (2000). “Alport syndrome: a review”. Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
  52. ↑ Amari F, Segawa K, Ando F (1994). “Lens coloboma and Alport-like glomerulonephritis”. Eur J Ophthalmol. 4 (3): 181–3. PMID 7819734.
  53. ↑ Govan JA (1983). “Ocular manifestations of Alport’s syndrome: a hereditary disorder of basement membranes?”. Br J Ophthalmol. 67 (8): 493–503. PMC 1040106. PMID 6871140.
  54. ↑ Savige J, Rana K, Tonna S, Buzza M, Dagher H, Wang YY (2003). “Thin basement membrane nephropathy”. Kidney Int. 64 (4): 1169–78. doi:10.1046/j.1523-1755.2003.00234.x. PMID 12969134. Unknown parameter |month= ignored (help)
  55. ↑ Hou P, Chen Y, Ding J, Li G, Zhang H (2007). “A novel mutation of COL4A3 presents a different contribution to Alport syndrome and thin basement membrane nephropathy”. Am. J. Nephrol. 27 (5): 538–44. doi:10.1159/000107666. PMID 17726307.
  56. ↑ Najafian B, Smith K, Lusco MA, Alpers CE, Fogo AB (October 2017). “AJKD Atlas of Renal Pathology: Nail-Patella Syndrome-Associated Nephropathy”. Am. J. Kidney Dis. 70 (4): e19–e20. doi:10.1053/j.ajkd.2017.08.001. PMID 28941488.
  57. ↑ Guidera KJ, Satterwhite Y, Ogden JA, Pugh L, Ganey T (1991). “Nail patella syndrome: a review of 44 orthopaedic patients”. J Pediatr Orthop. 11 (6): 737–42. PMID 1960197.
  58. ↑ Hughson MD, Puelles VG, Hoy WE, Douglas-Denton RN, Mott SA, Bertram JF (July 2014). “Hypertension, glomerular hypertrophy and nephrosclerosis: the effect of race”. Nephrol. Dial. Transplant. 29 (7): 1399–409. doi:10.1093/ndt/gft480. PMC 4071048. PMID 24327566.
  59. ↑ Lusco MA, Najafian B, Alpers CE, Fogo AB (April 2016). “AJKD Atlas of Renal Pathology: Cholesterol Emboli”. Am. J. Kidney Dis. 67 (4): e23–4. doi:10.1053/j.ajkd.2016.02.034. PMID 27012950.
  60. ↑ Wesson DE (June 2002). “The initiation and progression of sickle cell nephropathy”. Kidney Int. 61 (6): 2277–86. doi:10.1046/j.1523-1755.2002.00363.x. PMID 12028473.
  61. ↑ Lusco MA, Fogo AB, Najafian B, Alpers CE (December 2016). “AJKD Atlas of Renal Pathology: Thrombotic Microangiopathy”. Am. J. Kidney Dis. 68 (6): e33–e34. doi:10.1053/j.ajkd.2016.10.006. PMID 27884283.
  62. ↑ Jayakody Arachchillage D, Greaves M (2014). “The chequered history of the antiphospholipid syndrome”. Br J Haematol. 165 (5): 609–17. doi:10.1111/bjh.12848. PMID 24684307.
  63. ↑ Jayakody Arachchillage D, Greaves M (2014). “The chequered history of the antiphospholipid syndrome”. Br J Haematol. 165 (5): 609–17. doi:10.1111/bjh.12848. PMID 24684307.
  64. ↑ Popa A, Voinea L, Pop M, Stana D, Dascalu AM, Alexandrescu C; et al. (2008). “[Primary antiphospholipid syndrome]”. Oftalmologia. 52 (1): 13–7. PMID 18714484.

Template:WH

Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

Epidemiology and Demographics

Incidence

  • The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
  • In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.

Prevalence

  • The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
  • In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
  • The prevalence of [disease/malignancy] is estimated to be [number] cases annually.

Case-fatality rate/Mortality rate

  • In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%.
  • The case-fatality rate/mortality rate of [disease name] is approximately [number range].

Age

FSGS is considered a disease of the adult population (compared to minimal change disease which is more common among children). The median age of non-HIV associated FSGS leading to ESRD is 40-49 years in black adults and 70-79 in white and Asian adults.[1]

Gender

  • Men are more commonly affected by focal segmental glomerulosclerosis than women
  • The prevalence of FSGS male to female ratio is 1.5-2 to 1[1]

Race

FSGS is the most common primary renal cause of end-stage renal disease (ESRD) in whites and blacks, contributing to approximately 2% of ESRD.[1] FSGS is more common in blacks. It accounts for approximately 35% of nephrotic syndromes in all cases and approximately 50% of nephrotic syndrome in blacks.[2] FSGS seems to have a higher incidence in Blacks with a familial pattern of inheritance especially in Blacks with family history of ESKD.[3]

References

  1. ↑ 1.0 1.1 1.2 Kitiyakara C, Eggers P, Kopp JB (2004). “Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States”. Am J Kidney Dis. 44 (5): 815–25. PMID 15492947.
  2. ↑ Hogg R, Middleton J, Vehaskari VM (2007). “Focal segmental glomerulosclerosis–epidemiology aspects in children and adults”. Pediatr Nephrol. 22 (2): 183–6. doi:10.1007/s00467-006-0370-5. PMC 1764601. PMID 17151873.
  3. ↑ Reiser J, Nast CC, Alachkar N (2014). “Permeability factors in focal and segmental glomerulosclerosis”. Adv Chronic Kidney Dis. 21 (5): 417–21. doi:10.1053/j.ackd.2014.05.010. PMC 4149759. PMID 25168830 PMID 25168830 Check |pmid= value (help).

Template:WH Template:WS

Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

Common Risk Factors

Common risk factors in the development of focal segmental glomerulosclerosis include:[1][2]

  • Male gender
  • Black race
  • Family history
  • Heroin abuse
  • HIV
  • Drugs such as pamidronate
  • Solitary kidney
  • Obesity

References

  1. ↑ Bruggeman LA, Ross MD, Tanji N, Cara A, Dikman S, Gordon RE, Burns GC, D’Agati VD, Winston JA, Klotman ME, Klotman PE (November 2000). “Renal epithelium is a previously unrecognized site of HIV-1 infection”. J. Am. Soc. Nephrol. 11 (11): 2079–87. PMID 11053484.
  2. ↑ Wyatt CM, Klotman PE, D’Agati VD (November 2008). “HIV-associated nephropathy: clinical presentation, pathology, and epidemiology in the era of antiretroviral therapy”. Semin. Nephrol. 28 (6): 513–22. doi:10.1016/j.semnephrol.2008.08.005. PMC 2656916. PMID 19013322.


Template:WH Template:WS

Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]

Overview

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of focal segmental glomerulosclerosis usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
  • The symptoms of (disease name) typically develop ___ years after exposure to ___.
  • If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

Common complications of focal segmental glomerulosclerosis include:[1]

  • End stage renal disease
  • Malnutrition
  • Infections
  • Nephrotic syndrome

Prognosis

  • There are several clinical and pathological features which predict the outcome.
  • Factors which are associated with worse prognosis of focal segmental glomerulosclerosis include:[2][3]:
    • Black race
    • Increased degrees of proteinuria
    • renal insufficiency
    • Increased severity of interstitial fibrosis
    • Tubular atrophy in biopsy specimens

References

  1. ↑ Korbet SM (1999). “Clinical picture and outcome of primary focal segmental glomerulosclerosis”. Nephrol Dial Transplant. 14 Suppl 3: 68–73. PMID 10382985.
  2. ↑ “Focal Segmental Glomerulosclerosis in Nephrotic Adults: Presentation, Prognosis, and Response to Therapy of the Histologic Variants”.
  3. ↑ Sohal, DS; Prabhakar, SS (November 02, 2011). “Focal segmental glomerulosclerosis” (PDF). Interchopen. InTech. Retrieved 3 December 2013. Check date values in: |date= (help)


Template:WH Template:WS

Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

Template:Nephrology

Template:WH Template:WS

Looking for the patient version?

Back to the patient-friendly article

Imprint

Privacy Policy

Terms and Conditions

© 2026 MyEClinic – IFTM Institut für Telematik in der Medizin GmbH