Glomerular disease

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This page contains general information about Glomerular disease. For more information on specific types, please visit the pages on nephritic syndrome, 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 dieseases can be classified into several clinical and pathological syndromes as below:

Syndrome Disease
Acute nephritic syndromes
Nephrotic syndrome
Glomerular Deposition Diseases
Pulmonary-Renal Syndromes:
Basement Membrane Syndromes
Glomerular-Vascular Syndromes
Infectious Disease–Associated Syndromes

Also, glomerular diseases can be classified based on their clinical and urinary pattern in to below types:

Mild nephritc:

This category include mild nephritic sediment that is associated with less than half involvement of glomeruli.

Severe nephritic:

More severe clinical features such as edema, heavy proteinuria, hypertension, and/or renal failure may occur.

Nephrotic:

This syndrome is associated with heavy proteinuria and lipiduria.

Glomerular diseases also may classified by their presentation as below:

Glomerular hematuria:

1- Isolated hematuria

2- Glomerulonephritis (nephritic syndrome)

Proteinuria:

1-Isolated non-nephrotic proteinuria 

2- Nephrotic syndrome

Rapidly progressive glomerulonephritis

Glomerulonephritis

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.

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 Videos

Rapidly progressive glomerulonephritis

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Chronic glomerulonephritis

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Images

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

Images:


Differential Diagnosis

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]

Glomerular diseases Sub-entity History and Symtoms Laboratory Findings Pathology Comments
Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features
Acute Nephritic Syndromes Poststreptococcal Glomerulonephritis +/- + +/- +/- +/- +/- +/- +/-
  • Anti-DNA antibodies
  • Anti-C1q antibodies
  • Antineutrophil cytoplasmic antibodies (ANCA)
Hypercellular and inflamed glomeruli Immune complex GN, granular deposit Pathologic involvement correlates with the clinical findings
Renal disease due to Subacute Bacterial Endocarditis, or cardiac shunt (Atrioventricular)
  • History of infective endocarditis mostly due to S. aureus
  • Cardiac shunt
+/- + +/- +/- +/- +/- +/- +/-
  • Crescentic GN is the most common pathological features
  • Mesangial deposits,
  • Subendothelial deposits
  • Subepithelial "humps," in minority of cases
  •  Pauci-immune GN
Lupus Nephritis
  • History of SLE features
+/- + +/- +/- +/- +/- +/- +/-
  • Anti-C1q antibodies
  • Anti-dsDNA
Differs based on the disease classification Differs based on the disease classification Differs based on the disease classification, mostly immune complex GN, granular deposit Disease presentation is different based on lupus nephritis classifications
Antiglomerular Basement Membrane Disease (Goodpasture's syndrome)
  • Young adults
+ + + + + + - - Immune complex GN, Linear deposit
IgA Nephropathy + +/- + +/- + - + - Immune complex deposition
  • Crescent formation
Immune complex GN, granular deposite
ANCA Small-Vessel Vasculitis Granulomatosis with Polyangiitis (Wegener's)
  • Middle age male
+ + + +/- + - + - ANCA
  • Necrotizing and crescentic glomerulonephritis
 Pauci-immune GN
Microscopic Polyangiitis +/- + + + + + + - -  Pauci-immune GN
Churg-Strauss Syndrome +/- + + + + + + - Hypercellular and inflamed glomeruli (Crescent formation)-

(pauci-immune)

-  Pauci-immune GN
Membranoproliferative Glomerulonephritis + + + +/- + + - -
  • Immune complex deposition
Immune complex GN, granular deposite
Henoch-Schönlein purpura  + + + +/- + + - -
  • Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
Immune complex GN, granular deposite
Cryoglobulinemia Patients having cryoglobulinemia may have positive history of: Pulmonary symptoms:
  • Cough

Cutaneous symptoms:

Gastrointestinal symptoms:

  • Abdominal pain

General symptoms:

+/- + +/- + +/- +/- +/- +/- +/-
Nephrotic Syndrome Minimal Change Disease - + - + +/- + - +
  • Normal
-
Focal Segmental Glomerulosclerosis - + - + +/- + - + -
Membranous Glomerulonephritis - + - + +/- + - + Immune complex deposition Immune complex GN, granular deposite
Diabetic Nephropathy For more information on diabetes click here. - + - + +/- + - +
 Glomerular Deposition Diseases  Light Chain Deposition Disease[25]
  • 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
  • Many patients with light chain deposition disease progress to renal failure and dialysis
Renal Amyloidosis[26][27][28][29] - + - + +/- + - + -
Fibrillary-Immunotactoid Glomerulopathy[30] - +/- + +/- +/- +/- + +/- +/- -
  • 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[31][32][33] - + - + +/- + - + -
  • 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[34][35][36][37][38][39]
  • Positive family history
Auditary:

Occular problems:

  • Refractory Error
  • Arcus
  • Glaucoma
  • Vogt’s White Limbal Girdle
  • Band Keratopathy
  • Lenticonus
  • Spherophakia
  • Cataracts
  • Lens Coloboma
  • Anterior Lenticonus
  • Flecked Retinopathy of the Macula or Periphery
- + - + +/- + - + -
  • Early stage: unremarkable
-
Thin Basement Membrane Disease
  • Positive family history
- - + -/+ - -/+ - -/+ - - - Diffuse thinning of the glomerular basement membranes (GBM) -
Nail-Patella Syndrome[40]
  • Positive family history
  • Poorly developed fingernails, toenails, 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-Vascular Syndromes  Atherosclerotic Nephropathy
Hypertensive Nephrosclerosis
Cholesterol Emboli
Sickle Cell Disease
Thrombotic Microangiopathies
Antiphospholipid Antibody Syndrome 
 Infectious Disease–Associated Syndromes  Human Immunodeficiency Virus
Hepatitis B and C
Other Viruses
Syphilis
Leprosy
Malaria
Schistosomiasis
Other Parasites

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.
  25. 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.
  26. Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
  27. 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.
  28. 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.
  29. Pepys MB (2006). "Amyloidosis". Annu. Rev. Med. 57: 223–41. doi:10.1146/annurev.med.57.121304.131243. PMID 16409147.
  30. Korbet SM, Schwartz MM, Lewis EJ (March 1991). "Immunotactoid glomerulopathy". Am. J. Kidney Dis. 17 (3): 247–57. PMID 1996564.
  31. Alroy J, Sabnis S, Kopp JB (June 2002). "Renal pathology in Fabry disease". J. Am. Soc. Nephrol. 13 Suppl 2: S134–8. PMID 12068025.
  32. 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)
  33. 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)
  34. McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
  35. 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.
  36. 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.
  37. McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
  38. Amari F, Segawa K, Ando F (1994). "Lens coloboma and Alport-like glomerulonephritis". Eur J Ophthalmol. 4 (3): 181–3. PMID 7819734.
  39. 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.
  40. 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.

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