Lupus nephritis classification

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

Overview

Lupus nephritis may be classified according to the Renal Pathology Society/International Society of Nephrology (RPS/ISN) classification which includes minimal mesangial lupus nephritis (class I), mesangial proliferative lupus nephritis (class II), focal lupus nephritis (class III), diffuse lupus nephritis (class IV), lupus membranous nephropathy (class V) and advanced sclerosing lupus nephritis (class VI)

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.

Template:WH Template:WS

[pmid147179222-1] 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.

[pmid16861976-2] 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.

[pmid15569330-3] 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.

[pmid17264872-4] 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.

[pmid19374008-5] Markowitz GS, D'Agati VD (May 2009). "Classification of lupus nephritis". Curr. Opin. Nephrol. Hypertens. 18 (3): 220–5. PMID 19374008.

[pmid193740082-6] Markowitz GS, D'Agati VD (May 2009). "Classification of lupus nephritis". Curr. Opin. Nephrol. Hypertens. 18 (3): 220–5. PMID 19374008.

[pmid3656940-7] 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.

[pmid14747370-8] 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.

[pmid14717922-9] 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.

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