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'''This page contains general information about Glomerular disease. For more information on specific types, please visit the pages on [[hematuria]], [[proteinuria]], [[minimal change disease]], [[focal segmental glomerulosclerosis]], [[membranous glomerulonephritis]], [[IgA nephropathy]], [[rapidly progressive glomerulonephritis]], [[membranoproliferative glomerulonephritis]], [[Goodpasture syndrome]], [[Granulomatosis with polyangiitis]], [[Eosinophilic granulomatosis with polyangiitis]], and [[Microscopic polyangiitis]]. '''  
'''This page contains general information about Glomerular disease. For more information on specific types, please visit the pages on nephritic syndromes, nephrotic syndrome, Fabry's Disease, Amyloidosis, Pulmonary-Renal Syndromes (vasculitis), Thin Basement Membrane Disease, Alport's Syndrome, Anti-GBM Disease, Hypertensive Nephrosclerosis, and Subacute Bacterial Endocarditis.'''  


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Revision as of 15:05, 14 May 2018

This page contains general information about Glomerular disease. For more information on specific types, please visit the pages on nephritic syndromes, nephrotic syndrome, Fabry's Disease, Amyloidosis, Pulmonary-Renal Syndromes (vasculitis), Thin Basement Membrane Disease, Alport's Syndrome, Anti-GBM Disease, Hypertensive Nephrosclerosis, and Subacute Bacterial Endocarditis.

Glomerular disease
Acute Glomerulonephritis: Micro H&E high mag; an excellent example of acute exudative glomerulonephritis.
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Glomerular disease Main page

Glomerular disease patient information

Overview

Classification

1- Poststreptococcal Glomerulonephritis
2- Renal disease due to Subacute Bacterial Endocarditis, or cardiac shunt
3- Lupus nephritis
4- Goodpasture syndrome
5- IgA nephropathy
6- Granulomatosis with Polyangiitis (Wegener's)
7- Microscopic Polyangiitis
8- Churg-Strauss Syndrome
9- Membranoproliferative Glomerulonephritis
10- Henoch-Schönlein purpura
11- Cryoglobulinemia
12- Minimal change disease
13- Focal segmental glomerulosclerosis
14- Membranous glomerulonephritis
15- Diabetic Nephropathy
16- Glomerular deposition disease
17- Alport's Syndrome
18- Thin Basement Membrane Disease
19- Nail-Patella Syndrome
20- Hypertensive nephrosclerosis
21- Cholesterol Emboli
22- Sickle Cell Disease
23- Thrombotic Microangiopathies
24- Antiphospholipid Antibody Syndrome

Pathophysiology

Differential Diagnosis

Diagnosis

Prevention

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2], Syed Hassan A. Kazmi BSc, MD [3]

Overview

Glomerular disease is a condition that affects the glomerulus. It consists of different diseases with different clinical courses and treatment options. Glomerular disease can be isolated hematuria, isolated proteinuria; acute or chronic glomerulonephritis, and nephrotic or nephritic features of glomerulonephritis. The end stage of all of these diseases will be glomerulosclerosi swhich is characterized by fibrosis of the glomerulus, and end-stage renal disease.


Classification

Glomerular diseases can be classified by their presentation as below:

Acute nephritic syndromes

Poststreptococcal Glomerulonephritis

Subacute Bacterial Endocarditis

Lupus Nephritis

Antiglomerular Basement Membrane Disease

IgA Nephropathy

ANCA Small-Vessel Vasculitis

Granulomatosis with Polyangiitis (Wegener's)

Microscopic Polyangiitis

Churg-Strauss Syndrome

Membranoproliferative Glomerulonephritis

Mesangioproliferative Glomerulonephritis

Nephrotic syndrome

Minimal Change Disease

Focal Segmental Glomerulosclerosis

Membranous Glomerulonephritis

Diabetic Nephropathy

Glomerular Deposition Diseases

Light Chain Deposition Disease

Renal Amyloidosis

Fibrillary-Immunotactoid Glomerulopathy

Fabry's Disease

Pulmonary-Renal Syndromes:

Goodpasture's syndrome

granulomatosis with polyangiitis (Wegener's)

microscopic polyangiitis

Churg-Strauss vasculitis

Henoch-Schönlein purpura

cryoglobulinemia

Basement Membrane Syndromes

Anti-GBM Disease

Alport's Syndrome

Thin Basement Membrane Disease

Nail-Patella Syndrome

Glomerular-Vascular Syndromes

Atherosclerotic Nephropathy

Hypertensive Nephrosclerosis

Cholesterol Emboli

Sickle Cell Disease

Thrombotic Microangiopathies

Antiphospholipid Antibody Syndrome 

Infectious Disease–Associated Syndromes

Post-Streptococcal Glomerulonephritis

Subacute Bacterial Endocarditis

Human Immunodeficiency Virus

Hepatitis B and C

Other Viruses

Syphilis

Leprosy

Malaria

Schistosomiasis

Other Parasites

Glomerular hematuria:

1- Isolated hematuria

2- Glomerulonephritis (nephritic syndrome)

Glomerulonephritis which is inflammation of the glomeruli can be classified based on pathogenic type into three subtypes:

  • Anti-GBM disease: Linear deposit
    1. Goodpasture syndrome (renal and lung involvement)
    2. Renal involvement alone
    3. Lung involvement alone

Glomerulonephritis (nephritic syndrome) also may be classified based on disease course into acute or chronic nephritic syndrome; primary vs secondary causes; or systemic vs renal limited disease. For more information about nephritic syndrome classifications click here.

Proteinuria:

1-Isolated non-nephrotic proteinuria 

2- Nephrotic syndrome

Rapidly progressive glomerulonephritis

Pathophysiology

Microscopic Pathology

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

  • Glomerulonephritis: Micro H&E med mag; an excellent example of AGN with many neutrophils

    Glomerulonephritis: Micro H&E med mag; an excellent example of AGN with many neutrophils

  • Acute Glomerulonephritis: Micro H&E high mag; an excellent example of acute exudative glomerulonephritis.

    Acute Glomerulonephritis: Micro H&E high mag; an excellent example of acute exudative glomerulonephritis.


Glomerulonephritis Videos

Rapidly progressive glomerulonephritis

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Chronic glomerulonephritis

{{#ev:youtube|eA1vYarRAWo}}

Images

Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

  • This is a low-power photomicrograph of a saggital section of end stage chronic glomerulonephritis (GN). Note the marked thinning of the cortex (arrow).

    This is a low-power photomicrograph of a saggital section of end stage chronic glomerulonephritis (GN). Note the marked thinning of the cortex (arrow).

  • This is a higher-power photomicrograph of hyalinized glomeruli (arrows) and glomeruli with thick basement membranes.

    This is a higher-power photomicrograph of hyalinized glomeruli (arrows) and glomeruli with thick basement membranes.

  • This is a higher-power photomicrograph of hyalinized glomeruli (1) and glomeruli with thickened basement membranes (2).

    This is a higher-power photomicrograph of hyalinized glomeruli (1) and glomeruli with thickened basement membranes (2).

  • This is a photomicrograph of interstitial and vascular lesions in end stage renal disease.

    This is a photomicrograph of interstitial and vascular lesions in end stage renal disease.

  • This is an immunofluorescent photomicrograph of granular membranous immunofluorescence (immune complex disease). The antibody used for these studies was specific for IgG.

    This is an immunofluorescent photomicrograph of granular membranous immunofluorescence (immune complex disease). The antibody used for these studies was specific for IgG.

  • This is an electron micrograph of subepithelial granular electron dense deposits (arrows) which correspond to the granular immunofluorescence seen in the previous image.

    This is an electron micrograph of subepithelial granular electron dense deposits (arrows) which correspond to the granular immunofluorescence seen in the previous image.

  • This is a photomicrograph of a glomerulus from another case with acute poststreptococcal glomerulonephritis. In this case the immune complex glomerular disease is ongoing with necrosis and accumulation of neutrophils in the glomerulus.

    This is a photomicrograph of a glomerulus from another case with acute poststreptococcal glomerulonephritis. In this case the immune complex glomerular disease is ongoing with necrosis and accumulation of neutrophils in the glomerulus.

  • This immunofluorescent photomicrograph of a glomerulus from a case of acute poststreptococcal glomerulonephritis shows a granular immunofluorescence pattern consistent with immune complex disease. The primary antibody used for this staining was specific for IgG; however antibodies for complement would show a similar pattern.

    This immunofluorescent photomicrograph of a glomerulus from a case of acute poststreptococcal glomerulonephritis shows a granular immunofluorescence pattern consistent with immune complex disease. The primary antibody used for this staining was specific for IgG; however antibodies for complement would show a similar pattern.

  • This electron micrograph demonstrates scattered subepithelial dense deposits (arrows) and a polymorphonuclear leukocyte in the lumen.

    This electron micrograph demonstrates scattered subepithelial dense deposits (arrows) and a polymorphonuclear leukocyte in the lumen.

  • For comparison this is an immunofluorescent photomicrograph of a glomerulus from a patient with Goodpasture's syndrome. The linear (arrows) immunofluorescence is characteristic of Goodpasture's syndrome.

    For comparison this is an immunofluorescent photomicrograph of a glomerulus from a patient with Goodpasture's syndrome. The linear (arrows) immunofluorescence is characteristic of Goodpasture's syndrome.

Images:


Differential Diagnosis

Glomerular diseases Sub-entity Causes and associations History and Symtoms Laboratory Findings
Hyperlipidemia and hypercholesterolemia Nephrotic features Nephritic features ANCA Anti-glomerular basement membrane antibody (Anti-GBM antibody) Immune complex formation Light microscope Electron microscope Immunoflourescence pattern
History Pitting edema Hemeturia (pre-dominantly microscopic) Hypertension Hemoptysis Oliguria Peri-orbital edema
Acute Nephritic Syndromes Poststreptococcal Glomerulonephritis
Subacute Bacterial Endocarditis
Lupus Nephritis
Antiglomerular Basement Membrane Disease
IgA Nephropathy
ANCA Small-Vessel Vasculitis Granulomatosis with Polyangiitis (Wegener's)
Microscopic Polyangiitis
Churg-Strauss Syndrome
Membranoproliferative Glomerulonephritis
Mesangioproliferative Glomerulonephritis
Nephrotic Syndrome Minimal Change Disease
Focal Segmental Glomerulosclerosis
Membranous Glomerulonephritis
Diabetic Nephropathy
 Glomerular Deposition Diseases  Light Chain Deposition Disease
Renal Amyloidosis
Fibrillary-Immunotactoid Glomerulopathy
Fabry's Disease
Pulmonary-Renal Syndromes:  Goodpasture's syndrome
granulomatosis with polyangiitis (Wegener's)
microscopic polyangiitis
Churg-Strauss vasculitis
Henoch-Schönlein purpura 
cryoglobulinemia
 Basement Membrane Syndromes  Anti-GBM Disease
Alport's Syndrome
Thin Basement Membrane Disease
Nail-Patella Syndrome
 Glomerular-Vascular Syndromes  Atherosclerotic Nephropathy
Hypertensive Nephrosclerosis
Cholesterol Emboli
Sickle Cell Disease
Thrombotic Microangiopathies
Antiphospholipid Antibody Syndrome 
 Infectious Disease–Associated Syndromes  Post-Streptococcal Glomerulonephritis
Subacute Bacterial Endocarditis
Human Immunodeficiency Virus
Hepatitis B and C
Other Viruses
Syphilis
Leprosy
Malaria
Schistosomiasis
Other Parasites

Glomerulonephritis may be proliferative or non-proliferative and may be associated with nephrotic or nephritic features. The various types of glomerulonephritides should 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 glomerulonephritides:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24]

Glomerulonephritis Sub-entity Causes and associations History and Symtoms Laboratory Findings
Hyperlipidemia and hypercholesterolemia Nephrotic features Nephritic features ANCA Anti-glomerular basement membrane antibody (Anti-GBM antibody) Immune complex formation Light microscope Electron microscope Immunoflourescence pattern
History Pitting edema Hemeturia (pre-dominantly microscopic) Hypertension Hemoptysis Oliguria Peri-orbital edema
Non-proliferative Minimal change disease

+

-

-

-

+/-

-

+

+

-

-

-

-

  • Normal

-

Focal segmental glomerulosclerosis
  • Adults
 + - - - +/- - + + - - - - -
Membranous glomerulonephritis + - - - +/- - + + - - - + -
Proliferative IgA nephropathy +/- + + - + +/- - - + - - +
  • Crescent formation
 -
Rapidly progressive glomerulonephritis
  • Young adults
 +/- + + + + + - - + - + + + (Linear)
+/- + + + + + - - + - - + + (Granular)
+/- + + + + + - - + + (C-ANCA) - - - (pauci-immune) +/-
+/- + + + + + - - +

+ (C-ANCA)

- - - (pauci-immune) -
+/- + + + + + - - +

+ (P-ANCA)

- - - (pauci-immune) -
Membranoproliferative glomerulonephritis +/- + + + + + - + - - - + + (Granular)

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ Keskar V, Jamale TE, Kulkarni MJ, Kiggal Jagadish P, Fernandes G, Hase N (October 2013). "Minimal-change disease in adolescents and adults: epidemiology and therapeutic response". Clin Kidney J. 6 (5): 469–72. doi:10.1093/ckj/sft063. PMC 4438390. PMID 26064510.
  4. ↑ Chugh SS, Clement LC, Macé C (February 2012). "New insights into human minimal change disease: lessons from animal models". Am. J. Kidney Dis. 59 (2): 284–92. doi:10.1053/j.ajkd.2011.07.024. PMC 3253318. PMID 21974967.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ Wasserstein AG (April 1997). "Membranous glomerulonephritis". J. Am. Soc. Nephrol. 8 (4): 664–74. PMID 10495797.
  10. ↑ 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.
  11. ↑ Wyatt RJ, Julian BA (June 2013). "IgA nephropathy". N. Engl. J. Med. 368 (25): 2402–14. doi:10.1056/NEJMra1206793. PMID 23782179.
  12. ↑ He S, Wu Z (November 2011). "Gene-based Higher Criticism methods for large-scale exonic single-nucleotide polymorphism data". BMC Proc. 5 Suppl 9: S65. doi:10.1186/1753-6561-5-S9-S65. PMC 3287904. PMID 22373436.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ Bolton WK (November 1996). "Goodpasture's syndrome". Kidney Int. 50 (5): 1753–66. PMID 8914046.
  16. ↑ 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.
  17. ↑ 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.
  18. ↑ 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.
  19. ↑ 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.
  20. ↑ Cartin-Ceba R, Keogh KA, Specks U, Sethi S, Fervenza FC (September 2011). "Rituximab for the treatment of Churg-Strauss syndrome with renal involvement". Nephrol. Dial. Transplant. 26 (9): 2865–71. doi:10.1093/ndt/gfq852. PMC 3218640. PMID 21325353.
  21. ↑ 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.
  22. ↑ Pagnoux C (March 2008). "[Wegener's granulomatosis and microscopic polyangiitis]". Rev Prat (in French). 58 (5): 522–32. PMID 18524109.
  23. ↑ 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.
  24. ↑ 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.

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