Systemic lupus erythematosus classification scheme

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2], Raviteja Guddeti, M.B.B.S. [3], Kiran Singh, M.D. [4]

Overview

Lupus may be classified into several subtypes according to clinical features, including systemic lupus erythematosus, cutaneous lupus erythematosus, drug-induced lupus, and neonatal lupus. Systemic lupus erythematosus (SLE) itself may be classified into several subtypes based on dermatologic manifestations or glomerulonephritis. SLE may be classified according to dermatologic manifestations into 4 subtypes: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), chronic cutaneous lupus erythematosus (CCLE), and intermittent cutaneous lupus erythematosus (ICLE). SLE may be classified according to glomerulonephritis into 6 subtypes: 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

Classification of lupus based on clinical characteristics

Lupus may be classified into the following types based on clinical characteristics:^[1]

Classification of SLE based on dermatologic manifestations

SLE may be further classified according to dermatologic manifestations into 4 subtypes:^[1]^[2]^[3]

Acute cutaneous lupus erythematosus (ACLE)
Subacute cutaneous lupus erythematosus (SCLE)
Chronic cutaneous lupus erythematosus (CCLE)
Intermittent cutaneous lupus erythematosus (ICLE)

Subtypes	Manifestation/subclass
Acute cutaneous lupus erythematosus (ACLE)	Localized ACLE (i.e., malar rash, butterfly rash) Generalized ACLE Toxic epidermal necrolysis-like ACLE
Subacute cutaneous lupus erythematosus (SCLE)	Annular SCLE Papulosquamous SCLE Drug-induced SCLE Neonatal lupus (dermatitis manifestations) Less common subtypes: Erythrodermic Poikilodermatous Erythema multiforme-like (Rowell syndrome) Vesiculobullous annular SCLE
Chronic cutaneous lupus erythematosus (CCLE)	Discoid lupus erythematosus (DLE) Localized DLE Generalized DLE Hypertrophic DLE Lupus erythematosus tumidus (LE tumidus) Lupus profundus (also known as lupus panniculitis) Chilblain lupus erythematosus (chilblain LE) Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome)
Intermittent cutaneous lupus erythematosus (ICLE)	A consistent histopathologic feature of ACLE, SCLE, and discoid lupus erythematosus

Classification of SLE based on glomerulonephritis

SLE may be classified according to the degree of glomerulonephritis into 6 subtypes:^[4]^[5]^[6]

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)
Advanced sclerosing lupus nephritis (class VI)

Subtype	Manifestation/subclass
Minimal mesangial lupus nephritis (class I)	The earliest and mildest form of glomerular involvement Normal urinalysis, no or minimal proteinuria, and a normal serum creatinine
Mesangial proliferative lupus nephritis (class II)	Microscopic hematuria and/or proteinuria
Focal lupus nephritis (class III)	Hematuria and proteinuria May also have hypertension, a decreased glomerular filtration rate, and/or nephrotic syndrome
Diffuse lupus nephritis (class IV)	Most common and most severe form Hematuria and proteinuria Nephrotic syndrome, hypertension, and reduced glomerular filtration rate Hypocomplementemia (especially C3) and elevated anti-DNA levels, especially during active disease^[7]
Lupus membranous nephropathy (class V)	Hematuria and proteinuria Nephrotic syndrome, hypertension, and reduced glomerular filtration rate Hypocomplementemia
Advanced sclerosing lupus nephritis (class VI)	Slowly progressive renal dysfunction Proteinuria

References

↑ ^1.0 ^1.1 Lee HJ, Sinha AA (September 2006). "Cutaneous lupus erythematosus: understanding of clinical features, genetic basis, and pathobiology of disease guides therapeutic strategies". Autoimmunity. 39 (6): 433–44. doi:10.1080/08916930600886851. PMID 17060022.
↑ Lin JH, Dutz JP, Sontheimer RD, Werth VP (2007). "Pathophysiology of cutaneous lupus erythematosus". Clin Rev Allergy Immunol. 33 (1–2): 85–106. doi:10.1007/s12016-007-0031-x. PMID 18094949.
↑ Wallace DJ, Pistiner M, Nessim S, Metzger AL, Klinenberg JR (1992). "Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features". Semin. Arthritis Rheum. 21 (4): 221–6. PMID 1570517.
↑ Mannik M, Merrill CE, Stamps LD, Wener MH (2003). "Multiple autoantibodies form the glomerular immune deposits in patients with systemic lupus erythematosus". J. Rheumatol. 30 (7): 1495–504. PMID 12858447.
↑ Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Mejía JC, Aydintug AO, Chwalinska-Sadowska H, de Ramón E, Fernández-Nebro A, Galeazzi M, Valen M, Mathieu A, Houssiau F, Caro N, Alba P, Ramos-Casals M, Ingelmo M, Hughes GR (2003). "Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients". Medicine (Baltimore). 82 (5): 299–308. doi:10.1097/01.md.0000091181.93122.55. PMID 14530779.
↑ 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 (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.
↑ Lloyd W, Schur PH (1981). "Immune complexes, complement, and anti-DNA in exacerbations of systemic lupus erythematosus (SLE)". Medicine (Baltimore). 60 (3): 208–17. PMID 7231154.

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[pmid17060022-1] 1.0 ^1.1 Lee HJ, Sinha AA (September 2006). "Cutaneous lupus erythematosus: understanding of clinical features, genetic basis, and pathobiology of disease guides therapeutic strategies". Autoimmunity. 39 (6): 433–44. doi:10.1080/08916930600886851. PMID 17060022.

[pmid18094949-2] Lin JH, Dutz JP, Sontheimer RD, Werth VP (2007). "Pathophysiology of cutaneous lupus erythematosus". Clin Rev Allergy Immunol. 33 (1–2): 85–106. doi:10.1007/s12016-007-0031-x. PMID 18094949.

[pmid1570517-3] Wallace DJ, Pistiner M, Nessim S, Metzger AL, Klinenberg JR (1992). "Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features". Semin. Arthritis Rheum. 21 (4): 221–6. PMID 1570517.

[pmid12858447-4] Mannik M, Merrill CE, Stamps LD, Wener MH (2003). "Multiple autoantibodies form the glomerular immune deposits in patients with systemic lupus erythematosus". J. Rheumatol. 30 (7): 1495–504. PMID 12858447.

[pmid14530779-5] Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Mejía JC, Aydintug AO, Chwalinska-Sadowska H, de Ramón E, Fernández-Nebro A, Galeazzi M, Valen M, Mathieu A, Houssiau F, Caro N, Alba P, Ramos-Casals M, Ingelmo M, Hughes GR (2003). "Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients". Medicine (Baltimore). 82 (5): 299–308. doi:10.1097/01.md.0000091181.93122.55. PMID 14530779.

[pmid14717922-6] 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 (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.

[pmid7231154-7] Lloyd W, Schur PH (1981). "Immune complexes, complement, and anti-DNA in exacerbations of systemic lupus erythematosus (SLE)". Medicine (Baltimore). 60 (3): 208–17. PMID 7231154.

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