Lupus nephritis classification: Difference between revisions
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{{CMG}}; {{AE}} | {{CMG}}; {{AE}} | ||
==Overview== | ==Overview== | ||
Lupus nephritis may be classified according to The Renal Pathology Society/International Society of Nephrology (RPS/ISN) classification. | [[Lupus nephritis]] may be classified according to The Renal Pathology Society/International Society of Nephrology (RPS/ISN) classification. | ||
==Classification== | ==Classification== | ||
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=== Minimal mesangial lupus nephritis (class I) === | === Minimal mesangial lupus nephritis (class I) === | ||
* Very rare.<ref name="pmid147179222" /> | * Very rare.<ref name="pmid147179222" /> | ||
* No microscopic finding. | * No [[microscopic]] finding. | ||
* Identified on electron microscopy and immunofluorescence.<ref name="pmid147179222" /> | * Identified on [[electron microscopy]] and [[immunofluorescence]].<ref name="pmid147179222" /> | ||
=== Mesangial proliferative lupus nephritis (class II) === | === Mesangial proliferative lupus nephritis (class II) === | ||
* Microscopic Hematuria | * Microscopic [[Hematuria]] | ||
* Proteinuria | * [[Proteinuria]] | ||
* Hypercellularity in mesangium<ref name="pmid147179222" /> | * Hypercellularity in [[mesangium]]<ref name="pmid147179222" /> | ||
* Light microscopy shows subendothelial deposits or segmental scars<ref name="pmid147179222" /> | * Light microscopy shows subendothelial deposits or segmental scars<ref name="pmid147179222" /> | ||
* Immunofluorescence shows subepithelial or subendothelial deposits | * [[Immunofluorescence]] shows subepithelial or subendothelial deposits | ||
* Good prognosis | * Good prognosis | ||
=== Focal lupus nephritis (class III) === | === Focal lupus nephritis (class III) === | ||
* Less than 50% glomeruli involved | * Less than 50% glomeruli involved | ||
* Hematuria | * [[Hematuria]] | ||
* Proteinuria | * [[Proteinuria]] | ||
* Hypertension | * Hypertension | ||
* Crescent formation | * [[Crescentic glomerulonephritis|Crescent]] formation | ||
* Uniform involvement on immunofluorescence<ref name="pmid147179222" /><ref name="pmid3656940">{{cite journal |vauthors=Schwartz MM, Kawala KS, Corwin HL, Lewis EJ |title=The prognosis of segmental glomerulonephritis in systemic lupus erythematosus |journal=Kidney Int. |volume=32 |issue=2 |pages=274–9 |date=August 1987 |pmid=3656940 |doi= |url=}}</ref> | * Uniform involvement on [[immunofluorescence]]<ref name="pmid147179222" /><ref name="pmid3656940">{{cite journal |vauthors=Schwartz MM, Kawala KS, Corwin HL, Lewis EJ |title=The prognosis of segmental glomerulonephritis in systemic lupus erythematosus |journal=Kidney Int. |volume=32 |issue=2 |pages=274–9 |date=August 1987 |pmid=3656940 |doi= |url=}}</ref> | ||
* Subendothelial immune and mesangial deposits on electron microscopy<ref name="pmid147179222" /><ref name="pmid14747370">{{cite journal |vauthors=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 |title=The classification of glomerulonephritis in systemic lupus erythematosus revisited |journal=J. Am. Soc. Nephrol. |volume=15 |issue=2 |pages=241–50 |date=February 2004 |pmid=14747370 |doi= |url=}}</ref>. | * Subendothelial immune and [[Mesangial cell|mesangial]] deposits on electron microscopy<ref name="pmid147179222" /><ref name="pmid14747370">{{cite journal |vauthors=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 |title=The classification of glomerulonephritis in systemic lupus erythematosus revisited |journal=J. Am. Soc. Nephrol. |volume=15 |issue=2 |pages=241–50 |date=February 2004 |pmid=14747370 |doi= |url=}}</ref>. | ||
==== Subclasses on the basis of inflammatory activity of the lesions: ==== | ==== Subclasses on the basis of inflammatory activity of the lesions: ==== | ||
*Class III (A) called focal proliferative lupus nephritis: Just active lesions.<ref name="pmid14747370" /><ref name="pmid14717922">{{cite journal |vauthors=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 |title=The classification of glomerulonephritis in systemic lupus erythematosus revisited |journal=Kidney Int. |volume=65 |issue=2 |pages=521–30 |date=February 2004 |pmid=14717922 |doi=10.1111/j.1523-1755.2004.00443.x |url=}}</ref> | *Class III (A) called focal proliferative lupus [[Lupus nephritis|nephritis]]: Just active [[Lesion|lesions]].<ref name="pmid14747370" /><ref name="pmid14717922">{{cite journal |vauthors=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 |title=The classification of glomerulonephritis in systemic lupus erythematosus revisited |journal=Kidney Int. |volume=65 |issue=2 |pages=521–30 |date=February 2004 |pmid=14717922 |doi=10.1111/j.1523-1755.2004.00443.x |url=}}</ref> | ||
*Class III (A/C) called focal proliferative and sclerosing lupus nephritis: Active and chronic lesions<ref name="pmid14717922" /> | *Class III (A/C) called focal proliferative and sclerosing lupus [[nephritis]]: Active and chronic [[Lesion|lesions]]<ref name="pmid14717922" /> | ||
*Class III (C) called focal sclerosing lupus nephritis: Chronic inactive lesions with scarring. | *Class III (C) called focal sclerosing lupus nephritis: Chronic inactive lesions with scarring. | ||
=== Diffuse lupus nephritis (class IV) === | === Diffuse lupus nephritis (class IV) === | ||
* More than 50% glomeruli involved | * More than 50% [[Glomerulus|glomeruli]] involved | ||
* Proteinuria | * [[Proteinuria]] | ||
* Hematuria | * [[Hematuria]] | ||
* Nephrotic syndrome | * [[Nephrotic syndrome]] | ||
* Hypocomplementemia | * [[Hypocomplementemia]] | ||
* Increased anti-DNA antibodies | * Increased anti-DNA [[antibodies]] | ||
* Proliferative, necrotizing lesions | * Proliferative, necrotizing lesions | ||
* Crescent formation | * [[Crescentic glomerulonephritis|Crescent]] formation | ||
* Glomerular capillary wall thickening | * Glomerular capillary wall thickening | ||
* Monocytes, suppressor/cytotoxic T cells may be seen | * [[Monocytes]], suppressor/cytotoxic T cells may be seen | ||
* Subendothelial deposits on electron microscopy | * Subendothelial deposits on electron microscopy | ||
==== Subclasses on the basis of involvment of affected glomeruli: Segmental (S) or global (G) and by the inflammation ==== | ==== Subclasses on the basis of involvment of affected glomeruli: Segmental (S) or global (G) and by the inflammation ==== | ||
* Class IV-S (A), class IV-S with active lesions called diffuse segmental proliferative nephritis. | * Class IV-S (A), class IV-S with active lesions called diffuse segmental proliferative [[nephritis]]. | ||
* Class IV-G (A), class IV-G associated with active lesions called diffuse global proliferative nephritis. | * Class IV-G (A), class IV-G associated with active lesions called diffuse global proliferative [[nephritis]]. | ||
* Class IV-S (A/C),associated with active and chronic lesions called diffuse segmental proliferative and sclerosing nephritis. | * Class IV-S (A/C),associated with active and chronic [[Lesion|lesions]] called diffuse segmental proliferative and sclerosing nephritis. | ||
* Class IV-G (A/C), class IV-G with active and chronic lesions called diffuse global proliferative and sclerosing nephritis. | * Class IV-G (A/C), class IV-G with active and chronic [[Lesion|lesions]] called diffuse global proliferative and sclerosing nephritis. | ||
* Class IV-S (C), associated with chronic inactive lesions with scars called diffuse segmental sclerosing lupus nephritis. | * Class IV-S (C), associated with chronic inactive lesions with scars called diffuse segmental sclerosing lupus [[Lupus nephritis|nephritis]]. | ||
* Class IV-G (C), class IV-G with chronic inactive lesions with scars called diffuse global sclerosing lupus nephritis | * Class IV-G (C), class IV-G with chronic inactive lesions with scars called diffuse global sclerosing [[lupus nephritis]] | ||
=== Lupus membranous nephropathy (class V) === | === Lupus membranous nephropathy (class V) === | ||
* Nephrotic syndrome | * [[Nephrotic syndrome]] | ||
* Microscopic hematuria | * Microscopic [[hematuria]] | ||
* | * [[Hypertension]] | ||
* Light microscopy shows diffuse glomerular capillary wall thickening | * Light microscopy shows diffuse [[Glomerulus|glomerular]] capillary wall thickening | ||
* Immunofluorescence or electron microscopy shows subepithelial immune depositson | * [[Immunofluorescence]] or electron microscopy shows subepithelial immune depositson | ||
* May present no SLE features/Lab findings | * May present no [[SLE]] features/Lab findings | ||
=== Advanced sclerosing lupus nephritis (class VI) === | === Advanced sclerosing lupus nephritis (class VI) === | ||
* Proteinuria | * [[Proteinuria]] | ||
* Dull urine sediment | * Dull [[urine]] sediment | ||
* Greater than 90 percent of glomeruli involvement. | * Greater than 90 percent of [[Glomerulus|glomeruli]] involvement. | ||
* Presence of all advanced stages of chronic class III, IV, or V lupus nephritis. | * Presence of all advanced stages of chronic class III, IV, or V [[lupus nephritis]]. | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Lupus nephritis may be classified according to The Renal Pathology Society/International Society of Nephrology (RPS/ISN) classification.
Classification
The Renal Pathology Society/International Society of Nephrology (RPS/ISN) classification:[1][2][3][4][5][6]
Minimal mesangial lupus nephritis (class I)
- Very rare.[1]
- No microscopic finding.
- Identified on electron microscopy and immunofluorescence.[1]
Mesangial proliferative lupus nephritis (class II)
- Microscopic Hematuria
- Proteinuria
- Hypercellularity in mesangium[1]
- Light microscopy shows subendothelial deposits or segmental scars[1]
- Immunofluorescence shows subepithelial or subendothelial deposits
- Good prognosis
Focal lupus nephritis (class III)
- Less than 50% glomeruli involved
- Hematuria
- Proteinuria
- Hypertension
- Crescent formation
- Uniform involvement on immunofluorescence[1][7]
- Subendothelial immune and mesangial deposits on electron microscopy[1][8].
Subclasses on the basis of inflammatory activity of the lesions:
- Class III (A) called focal proliferative lupus nephritis: Just active lesions.[8][9]
- Class III (A/C) called focal proliferative and sclerosing lupus nephritis: Active and chronic lesions[9]
- Class III (C) called focal sclerosing lupus nephritis: Chronic inactive lesions with scarring.
Diffuse lupus nephritis (class IV)
- More than 50% glomeruli involved
- Proteinuria
- Hematuria
- Nephrotic syndrome
- Hypocomplementemia
- Increased anti-DNA antibodies
- Proliferative, necrotizing lesions
- Crescent formation
- Glomerular capillary wall thickening
- Monocytes, suppressor/cytotoxic T cells may be seen
- Subendothelial deposits on electron microscopy
Subclasses on the basis of involvment of affected glomeruli: Segmental (S) or global (G) and by the inflammation
- Class IV-S (A), class IV-S with active lesions called diffuse segmental proliferative nephritis.
- Class IV-G (A), class IV-G associated with active lesions called diffuse global proliferative nephritis.
- Class IV-S (A/C),associated with active and chronic lesions called diffuse segmental proliferative and sclerosing nephritis.
- Class IV-G (A/C), class IV-G with active and chronic lesions called diffuse global proliferative and sclerosing nephritis.
- Class IV-S (C), associated with chronic inactive lesions with scars called diffuse segmental sclerosing lupus nephritis.
- Class IV-G (C), class IV-G with chronic inactive lesions with scars called diffuse global sclerosing lupus nephritis
Lupus membranous nephropathy (class V)
- Nephrotic syndrome
- Microscopic hematuria
- Hypertension
- Light microscopy shows diffuse glomerular capillary wall thickening
- Immunofluorescence or electron microscopy shows subepithelial immune depositson
- May present no SLE features/Lab findings
Advanced sclerosing lupus nephritis (class VI)
- Proteinuria
- Dull urine sediment
- Greater than 90 percent of glomeruli involvement.
- Presence of all advanced stages of chronic class III, IV, or V lupus nephritis.
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 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.
- ↑ Furness PN, Taub N (August 2006). "Interobserver reproducibility and application of the ISN/RPS classification of lupus nephritis-a UK-wide study". Am. J. Surg. Pathol. 30 (8): 1030–5. PMID 16861976.
- ↑ Yokoyama H, Wada T, Hara A, Yamahana J, Nakaya I, Kobayashi M, Kitagawa K, Kokubo S, Iwata Y, Yoshimoto K, Shimizu K, Sakai N, Furuichi K (December 2004). "The outcome and a new ISN/RPS 2003 classification of lupus nephritis in Japanese". Kidney Int. 66 (6): 2382–8. doi:10.1111/j.1523-1755.2004.66027.x. PMID 15569330.
- ↑ Markowitz GS, D'Agati VD (March 2007). "The ISN/RPS 2003 classification of lupus nephritis: an assessment at 3 years". Kidney Int. 71 (6): 491–5. doi:10.1038/sj.ki.5002118. PMID 17264872.
- ↑ Markowitz GS, D'Agati VD (May 2009). "Classification of lupus nephritis". Curr. Opin. Nephrol. Hypertens. 18 (3): 220–5. PMID 19374008.
- ↑ Markowitz GS, D'Agati VD (May 2009). "Classification of lupus nephritis". Curr. Opin. Nephrol. Hypertens. 18 (3): 220–5. PMID 19374008.
- ↑ Schwartz MM, Kawala KS, Corwin HL, Lewis EJ (August 1987). "The prognosis of segmental glomerulonephritis in systemic lupus erythematosus". Kidney Int. 32 (2): 274–9. PMID 3656940.
- ↑ 8.0 8.1 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". J. Am. Soc. Nephrol. 15 (2): 241–50. PMID 14747370.
- ↑ 9.0 9.1 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.