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__NOTOC__
{{Systemic lupus erythematosus}}
{{Systemic lupus erythematosus}}


{{CMG}}; {{AE}} {{RT}}
{{CMG}}; {{AE}} {{MIR}}, {{RT}}, {{KS}}


==Overview==
==Overview==


Lupus is a chronic [[Autoimmunity|autoimmune disease]] in which the body's own defense system attacks otherwise healthy tissue.  
[[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).
Clinically, it can affect multiple organ systems including the heart, skin, joints, kidneys and nervous system.


==Classification==  
==Classification==


There are several types of lupus; in general, when the word lupus alone is used, reference is to systemic lupus erythematosus, as discussed in this article. Other types include:<ref name="Andrews">James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Chapter 8. ISBN 0721629210.</ref> Other types include:  
=== Classification of lupus 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)
* [[Cutaneous lupus erythematosus]] (CLE)
* [[Drug-induced lupus erythematosus|Drug-induced lupus]]
* [[Neonatal lupus]]
=== Classification of SLE based on dermatologic manifestations ===
[[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)
* Subacute cutaneous lupus erythematosus (SCLE)
* Chronic cutaneous lupus erythematosus (CCLE)
* Intermittent cutaneous lupus erythematosus (ICLE)
{| class="wikitable"
! style="background: #4479BA; color: #FFFFFF; " align="center" |Subtypes
! style="background: #4479BA; color: #FFFFFF; " align="center" |Manifestation/subclass
|-
| style="background: #DCDCDC; " |Acute cutaneous lupus erythematosus (ACLE)
|
* Localized ACLE (i.e., [[malar rash]], [[butterfly rash]])
* Generalized ACLE
* [[Toxic epidermal necrolysis]]-like ACLE
|-
| style="background: #DCDCDC; " |Subacute cutaneous lupus erythematosus (SCLE)
|
* Annular SCLE
* [[Papulosquamous|Papulosquamous SCLE]]
* [[Drug-induced]] SCLE
* [[Neonatal]] lupus ([[dermatitis]] manifestations)
* Less common subtypes:
** [[Erythroderma|Erythrodermic]]
** Poikilodermatous
** [[Erythema multiforme|Erythema multiforme-like]] (Rowell syndrome)
** Vesiculobullous annular SCLE
|-
| style="background: #DCDCDC; " |Chronic cutaneous lupus erythematosus (CCLE)
|
* [[Discoid lupus erythematosus]] (DLE)
** [[Localized oedema|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)
|-
| style="background: #DCDCDC; " |Intermittent cutaneous lupus erythematosus (ICLE)
|
* A consistent [[histopathologic]] feature of ACLE, SCLE, and [[discoid lupus erythematosus]]
|}


*Chronic cutaneous lupus erythematosus
=== Classification of SLE based on glomerulonephritis ===
**[[Discoid lupus erythematosus]], a skin disorder that causes a red, raised rash on the face and scalp. Discoid lupus occasionally (1–5%) develops into SLE.<ref>[http://www.skinsite.com/info_discoid_lupus_erythematosus.htm Discoid Lupus Erythematosus]</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>
***Localized discoid lupus erythematosus
* Minimal mesangial [[lupus nephritis]] (class I)
***Generalized discoid lupus erythematosus
* Mesangial proliferative [[lupus nephritis]] (class II)
***Childhood discoid lupus erythematosus
* Focal [[lupus nephritis]] (class III)
**Chilblain lupus erythematosus (Hutchinson)
* Diffuse [[lupus nephritis]] (class IV)
**Lupus erythematosus-lichen planus overlap syndrome
* [[Membranous nephropathy|Lupus membranous nephropathy]] (class V)
**Lupus erythematosus panniculitis (Lupus erythematosus profundus)
* Advanced sclerosing [[lupus nephritis]] (class VI)
**Subacute cutaneous lupus erythematosus, which causes nonscarring skin [[lesions]] on patches of skin exposed to sunlight.<ref name=niams>{{cite web |url=http://www.niams.nih.gov/Health_Info/Lupus/default.asp |title=Handout on Health: Systemic Lupus Erythematosus |accessdate=2007-11-23 |month=August | year=2003 |work=The National Institute of Arthritis and Musculoskeletal and Skin Diseases |publisher=National Institutes of Health}}</ref>
{| class="wikitable"
**Tumid lupus erythematosus
! style="background: #4479BA; color: #FFFFFF; " align="center" |Subtype
**Verrucous lupus erythematosus (Hypertrophic lupus erythematosus)
! style="background: #4479BA; color: #FFFFFF; " align="center" |Manifestation/subclass
*Neonatal lupus erythematosus, a rare disease affecting babies born to women with SLE, [[Sjögren's syndrome]], or sometimes no autoimmune disorder. It is theorized that maternal antibodies attack the fetus, causing skin rash; liver problems; low blood counts, which gradually fade; and heart block, leading to [[bradycardia]].<ref name=niams/>
|-
*Childhood systemic lupus erythematosus, the pediatric variant of systemic lupus erythematosus.
| style="background: #DCDCDC; " |Minimal mesangial [[lupus nephritis]] (class I)
*[[Drug-induced lupus erythematosus]], a drug-induced form of SLE; this type of lupus can occur equally in either sex.
|
*[[Lupus nephritis]], an inflammation of the [[kidney]]s caused by SLE.
* The earliest and mildest form of [[Glomerulus|glomerular]] involvement
*Complement deficiency syndromes
* Normal [[urinalysis]], no or minimal [[proteinuria]], and a normal serum [[creatinine]]
|-
| style="background: #DCDCDC; " |Mesangial proliferative [[lupus nephritis]] (class II)
|
* [[hematuria|Microscopic hematuria]] and/or [[proteinuria]]
|-
| style="background: #DCDCDC; " |Focal [[lupus nephritis]] (class III)
|
* [[Hematuria]] and [[proteinuria]]
* May also have [[hypertension]], a decreased [[Glomerular filtration rate|glomerular filtration rate,]] and/or [[nephrotic syndrome]]
|-
| style="background: #DCDCDC; " |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<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>
|-
| style="background: #DCDCDC; " |Lupus membranous [[nephropathy]] (class V) 
|
* [[Hematuria]] and [[proteinuria]]
* [[Nephrotic syndrome]], [[hypertension]], and reduced [[glomerular filtration rate]]
* [[Hypocomplementemia]]
|-
| style="background: #DCDCDC; " |Advanced sclerosing [[lupus nephritis]] (class VI)
|
* Slowly progressive [[renal dysfunction]]
* [[Proteinuria]]
|}


==References==
==References==
{{reflist|2}}
{{reflist|2}}


 
[[Category:Arthritis]]
[[Category:Dermatology]]
[[Category:Diseases involving the fasciae]]
[[Category:Rheumatology]]
[[Category:Nephrology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]


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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]

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]

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)
  • Most common and most severe form
Lupus membranous nephropathy (class V) 
Advanced sclerosing lupus nephritis (class VI)

References

  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.
  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.
  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.
  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.
  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.
  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.
  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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