Systemic lupus erythematosus classification scheme: Difference between revisions
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{{Systemic lupus erythematosus}} | {{Systemic lupus erythematosus}} | ||
{{CMG}}; {{AE}} {{RT}} {{KS}} | {{CMG}}; {{AE}} {{MIR}}, {{RT}}, {{KS}} | ||
==Overview== | ==Overview== | ||
Lupus may be classified into several subtypes according to clinical features | [[Lupus]] may be classified into several subtypes according to clinical features, including [[systemic lupus erythematosus]], [[cutaneous lupus erythematosus]], [[Drug-induced lupus erythematosus|drug-induced lupus]], and [[Neonatal lupus erythematosus|neonatal lupus]]. [[Systemic lupus erythematosus]] ([[SLE]]) itself may be classified into several subtypes based on [[Dermatology|dermatologic]] manifestations or [[glomerulonephritis]]. [[SLE]] may be classified according to [[Dermatology|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== | ||
=== Classification based on clinical characteristics === | === Classification of lupus based on clinical characteristics === | ||
Lupus may be classified into the following types based on clinical characteristics: | Lupus may be classified into the following types based on clinical characteristics:<ref name="pmid17060022">{{cite journal |vauthors=Lee HJ, Sinha AA |title=Cutaneous lupus erythematosus: understanding of clinical features, genetic basis, and pathobiology of disease guides therapeutic strategies |journal=Autoimmunity |volume=39 |issue=6 |pages=433–44 |date=September 2006 |pmid=17060022 |doi=10.1080/08916930600886851 |url=}}</ref> | ||
* Systemic lupus erythematosus (SLE) | * [[Systemic lupus erythematosus]] (SLE) | ||
* Cutaneous lupus erythematosus (CLE) | * [[Cutaneous lupus erythematosus]] (CLE) | ||
* Drug-induced lupus | * [[Drug-induced lupus erythematosus|Drug-induced lupus]] | ||
* Neonatal lupus | * [[Neonatal lupus]] | ||
=== Classification of SLE based on dermatologic manifestations === | |||
SLE may be further classified according to dermatologic manifestations into 4 subtypes:<ref name="pmid17060022">{{cite journal |vauthors=Lee HJ, Sinha AA |title=Cutaneous lupus erythematosus: understanding of clinical features, genetic basis, and pathobiology of disease guides therapeutic strategies |journal=Autoimmunity |volume=39 |issue=6 |pages=433–44 |year=2006 |pmid=17060022 |doi=10.1080/08916930600886851 |url=}}</ref><ref name="pmid18094949">{{cite journal |vauthors=Lin JH, Dutz JP, Sontheimer RD, Werth VP |title=Pathophysiology of cutaneous lupus erythematosus |journal=Clin Rev Allergy Immunol |volume=33 |issue=1-2 |pages=85–106 |year=2007 |pmid=18094949 |doi=10.1007/s12016-007-0031-x |url=}}</ref><ref name="pmid1570517">{{cite journal |vauthors=Wallace DJ, Pistiner M, Nessim S, Metzger AL, Klinenberg JR |title=Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features |journal=Semin. Arthritis Rheum. |volume=21 |issue=4 |pages=221–6 |year=1992 |pmid=1570517 |doi= |url=}}</ref> | [[SLE]] may be further classified according to [[Dermatology|dermatologic]] manifestations into 4 subtypes:<ref name="pmid17060022">{{cite journal |vauthors=Lee HJ, Sinha AA |title=Cutaneous lupus erythematosus: understanding of clinical features, genetic basis, and pathobiology of disease guides therapeutic strategies |journal=Autoimmunity |volume=39 |issue=6 |pages=433–44 |year=2006 |pmid=17060022 |doi=10.1080/08916930600886851 |url=}}</ref><ref name="pmid18094949">{{cite journal |vauthors=Lin JH, Dutz JP, Sontheimer RD, Werth VP |title=Pathophysiology of cutaneous lupus erythematosus |journal=Clin Rev Allergy Immunol |volume=33 |issue=1-2 |pages=85–106 |year=2007 |pmid=18094949 |doi=10.1007/s12016-007-0031-x |url=}}</ref><ref name="pmid1570517">{{cite journal |vauthors=Wallace DJ, Pistiner M, Nessim S, Metzger AL, Klinenberg JR |title=Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features |journal=Semin. Arthritis Rheum. |volume=21 |issue=4 |pages=221–6 |year=1992 |pmid=1570517 |doi= |url=}}</ref> | ||
* Acute cutaneous lupus erythematosus (ACLE) | * Acute cutaneous lupus erythematosus (ACLE) | ||
* Subacute cutaneous lupus erythematosus (SCLE) | * Subacute cutaneous lupus erythematosus (SCLE) | ||
| Line 23: | Line 23: | ||
* Intermittent cutaneous lupus erythematosus (ICLE) | * Intermittent cutaneous lupus erythematosus (ICLE) | ||
{| class="wikitable" | {| class="wikitable" | ||
!Subtypes | ! style="background: #4479BA; color: #FFFFFF; " align="center" |Subtypes | ||
!Manifestation/subclass | ! style="background: #4479BA; color: #FFFFFF; " align="center" |Manifestation/subclass | ||
|- | |- | ||
|Acute cutaneous lupus erythematosus (ACLE) | | style="background: #DCDCDC; " |Acute cutaneous lupus erythematosus (ACLE) | ||
| | | | ||
* Localized ACLE ( | * Localized ACLE (i.e., [[malar rash]], [[butterfly rash]]) | ||
* Generalized ACLE | * Generalized ACLE | ||
* Toxic epidermal necrolysis-like ACLE | * [[Toxic epidermal necrolysis]]-like ACLE | ||
|- | |- | ||
|Subacute cutaneous lupus erythematosus (SCLE) | | style="background: #DCDCDC; " |Subacute cutaneous lupus erythematosus (SCLE) | ||
| | | | ||
* Annular SCLE | * Annular SCLE | ||
* [[Papulosquamous|Papulosquamous SCLE]] | * [[Papulosquamous|Papulosquamous SCLE]] | ||
* Drug-induced SCLE | * [[Drug-induced]] SCLE | ||
* [[Neonatal]] lupus ([[dermatitis]] manifestations) | * [[Neonatal]] lupus ([[dermatitis]] manifestations) | ||
* Less common subtypes: | * Less common subtypes: | ||
** Erythrodermic | ** [[Erythroderma|Erythrodermic]] | ||
** Poikilodermatous | ** Poikilodermatous | ||
** Erythema multiforme-like (Rowell syndrome) | ** [[Erythema multiforme|Erythema multiforme-like]] (Rowell syndrome) | ||
** Vesiculobullous annular SCLE | ** Vesiculobullous annular SCLE | ||
|- | |- | ||
|Chronic cutaneous lupus erythematosus (CCLE) | | style="background: #DCDCDC; " |Chronic cutaneous lupus erythematosus (CCLE) | ||
| | | | ||
* [[Discoid lupus erythematosus]] (DLE) | * [[Discoid lupus erythematosus]] (DLE) | ||
** Localized DLE | ** [[Localized oedema|Localized]] DLE | ||
** Generalized DLE | ** Generalized DLE | ||
** Hypertrophic DLE | ** [[Hypertrophic]] DLE | ||
* Lupus erythematosus tumidus (LE tumidus) | * Lupus erythematosus tumidus (LE tumidus) | ||
* Lupus profundus (also known as lupus panniculitis) | * Lupus profundus (also known as lupus panniculitis) | ||
* [[Chilblain]] lupus erythematosus (chilblain LE) | * [[Chilblain]] lupus erythematosus (chilblain LE) | ||
* Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome) | * Lichenoid cutaneous lupus erythematosus-[[lichen planus]] overlap syndrome (LE-LP overlap syndrome) | ||
|- | |- | ||
|Intermittent cutaneous lupus erythematosus (ICLE) | | style="background: #DCDCDC; " |Intermittent cutaneous lupus erythematosus (ICLE) | ||
| | | | ||
* A consistent [[histopathologic]] feature of ACLE, SCLE, and discoid lupus erythematosus | * A consistent [[histopathologic]] feature of ACLE, SCLE, and [[discoid lupus erythematosus]] | ||
|} | |} | ||
=== SLE | === Classification of SLE based on glomerulonephritis === | ||
SLE may be classified according to [[glomerulonephritis]] into 6 subtypes:<ref name="pmid12858447">{{cite journal |vauthors=Mannik M, Merrill CE, Stamps LD, Wener MH |title=Multiple autoantibodies form the glomerular immune deposits in patients with systemic lupus erythematosus |journal=J. Rheumatol. |volume=30 |issue=7 |pages=1495–504 |year=2003 |pmid=12858447 |doi= |url=}}</ref><ref name="pmid14530779">{{cite journal |vauthors=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 |title=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 |journal=Medicine (Baltimore) |volume=82 |issue=5 |pages=299–308 |year=2003 |pmid=14530779 |doi=10.1097/01.md.0000091181.93122.55 |url=}}</ref><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 |year=2004 |pmid=14717922 |doi=10.1111/j.1523-1755.2004.00443.x |url=}}</ref> | SLE may be classified according to the degree of [[glomerulonephritis]] into 6 subtypes:<ref name="pmid12858447">{{cite journal |vauthors=Mannik M, Merrill CE, Stamps LD, Wener MH |title=Multiple autoantibodies form the glomerular immune deposits in patients with systemic lupus erythematosus |journal=J. Rheumatol. |volume=30 |issue=7 |pages=1495–504 |year=2003 |pmid=12858447 |doi= |url=}}</ref><ref name="pmid14530779">{{cite journal |vauthors=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 |title=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 |journal=Medicine (Baltimore) |volume=82 |issue=5 |pages=299–308 |year=2003 |pmid=14530779 |doi=10.1097/01.md.0000091181.93122.55 |url=}}</ref><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 |year=2004 |pmid=14717922 |doi=10.1111/j.1523-1755.2004.00443.x |url=}}</ref> | ||
* Minimal mesangial lupus nephritis (class I) | * Minimal mesangial [[lupus nephritis]] (class I) | ||
* Mesangial proliferative lupus nephritis (class II) | * Mesangial proliferative [[lupus nephritis]] (class II) | ||
* Focal lupus nephritis (class III) | * Focal [[lupus nephritis]] (class III) | ||
* Diffuse lupus nephritis (class IV) | * Diffuse [[lupus nephritis]] (class IV) | ||
* Lupus membranous nephropathy (class V) | * [[Membranous nephropathy|Lupus membranous nephropathy]] (class V) | ||
* Advanced sclerosing lupus nephritis (class VI) | * Advanced sclerosing [[lupus nephritis]] (class VI) | ||
{| class="wikitable" | {| class="wikitable" | ||
!Subtype | ! style="background: #4479BA; color: #FFFFFF; " align="center" |Subtype | ||
!Manifestation | ! style="background: #4479BA; color: #FFFFFF; " align="center" |Manifestation/subclass | ||
|- | |- | ||
|Minimal mesangial lupus nephritis (class I) | | style="background: #DCDCDC; " |Minimal mesangial [[lupus nephritis]] (class I) | ||
| | | | ||
* The earliest and mildest form of [[Glomerulus|glomerular]] involvement | * The earliest and mildest form of [[Glomerulus|glomerular]] involvement | ||
* Normal [[urinalysis]], no or minimal [[proteinuria]], and a normal serum [[creatinine]] | * Normal [[urinalysis]], no or minimal [[proteinuria]], and a normal serum [[creatinine]] | ||
|- | |- | ||
|Mesangial proliferative lupus nephritis (class II) | | style="background: #DCDCDC; " |Mesangial proliferative [[lupus nephritis]] (class II) | ||
| | | | ||
* | * [[hematuria|Microscopic hematuria]] and/or [[proteinuria]] | ||
|- | |- | ||
|Focal lupus nephritis (class III) | | style="background: #DCDCDC; " |Focal [[lupus nephritis]] (class III) | ||
| | | | ||
* [[Hematuria]] and [[proteinuria]] | * [[Hematuria]] and [[proteinuria]] | ||
* May also have [[hypertension]], a decreased [[Glomerular filtration rate|glomerular filtration rate,]] and/or [[nephrotic syndrome]] | * May also have [[hypertension]], a decreased [[Glomerular filtration rate|glomerular filtration rate,]] and/or [[nephrotic syndrome]] | ||
|- | |- | ||
|Diffuse lupus nephritis (class IV) | | style="background: #DCDCDC; " |Diffuse [[lupus nephritis]] (class IV) | ||
| | | | ||
* Most common and most severe form | * Most common and most severe form | ||
| Line 92: | Line 92: | ||
* [[Hematuria]] and [[proteinuria]] | * [[Hematuria]] and [[proteinuria]] | ||
* [[Nephrotic syndrome]], [[hypertension]], and reduced [[glomerular filtration rate]] | * [[Nephrotic syndrome]], [[hypertension]], and reduced [[glomerular filtration rate]] | ||
* [[Hypocomplementemia]] (especially C3) and elevated anti-DNA levels, especially during active | * [[Hypocomplementemia]] (especially C3) and elevated anti-DNA levels, especially during active disease<ref name="pmid7231154">{{cite journal |vauthors=Lloyd W, Schur PH |title=Immune complexes, complement, and anti-DNA in exacerbations of systemic lupus erythematosus (SLE) |journal=Medicine (Baltimore) |volume=60 |issue=3 |pages=208–17 |year=1981 |pmid=7231154 |doi= |url=}}</ref> | ||
|- | |- | ||
|Lupus membranous [[nephropathy]] (class V) | | style="background: #DCDCDC; " |Lupus membranous [[nephropathy]] (class V) | ||
| | | | ||
* [[Hematuria]] and [[proteinuria]] | * [[Hematuria]] and [[proteinuria]] | ||
| Line 100: | Line 100: | ||
* [[Hypocomplementemia]] | * [[Hypocomplementemia]] | ||
|- | |- | ||
|Advanced sclerosing lupus nephritis (class VI) | | style="background: #DCDCDC; " |Advanced sclerosing [[lupus nephritis]] (class VI) | ||
| | | | ||
* Slowly progressive [[renal dysfunction]] | * Slowly progressive [[renal dysfunction]] | ||
Latest revision as of 14:22, 26 June 2018
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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]
- Systemic lupus erythematosus (SLE)
- Cutaneous lupus erythematosus (CLE)
- Drug-induced lupus
- Neonatal lupus
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) |
|
| Subacute cutaneous lupus erythematosus (SCLE) |
|
| Chronic cutaneous lupus erythematosus (CCLE) |
|
| Intermittent cutaneous lupus erythematosus (ICLE) |
|
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) |
|
| 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) |
|
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.