Systemic lupus erythematosus classification scheme: Difference between revisions

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==Overview==
==Overview==


Lupus may be classified into several subtypes according to clinical features which include: 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 glomerulonephritis and dermatologic manifestation. 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).
Lupus may be classified into several subtypes according to clinical features which include: 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 [[glomerulonephritis]] and [[Dermatology|dermatologic]] manifestation. 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==
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Based on this classification, SLE is a subtype of lupus that can be classified into different subclasses as well. CLE can be presented with or without systemic symptoms of the disease.
Based on this classification, SLE is a subtype of lupus that can be classified into different subclasses as well. CLE can be presented with or without systemic symptoms of the disease.


Systemic lupus erythematosus (SLE) may be classified into several subtypes based on glomerulonephritis and dermatologic manifestation.
Systemic lupus erythematosus (SLE) may be classified into several subtypes based on [[glomerulonephritis]] and [[Dermatology|dermatologic]] manifestation.


=== SLE classification based on dermatologic manifestations ===
=== SLE classification based on dermatologic manifestations ===
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|Acute cutaneous lupus erythematosus (ACLE)
|Acute cutaneous lupus erythematosus (ACLE)
|
|
* Localized ACLE (ie, malar rash, butterfly rash)
* Localized ACLE (ie, [[malar rash]], [[butterfly rash]])
* Generalized ACLE
* Generalized ACLE
* Toxic epidermal necrolysis-like ACLE
* Toxic epidermal necrolysis-like ACLE
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|
|
* Annular SCLE
* Annular SCLE
* 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
** Erythrodermic
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|Chronic cutaneous lupus erythematosus (CCLE)
|Chronic cutaneous lupus erythematosus (CCLE)
|
|
* Discoid lupus erythematosus (DLE)
* [[Discoid lupus erythematosus]] (DLE)
** Localized DLE
** Localized DLE
** Generalized DLE
** Generalized DLE
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* 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)
|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 based on glomerulonephritis ===
=== SLE classification based on [[glomerulonephritis]] ===
SLE may be classified according to glomerulonephritis into 6 subtypes:
SLE may be classified according to [[glomerulonephritis]] into 6 subtypes:
* Minimal mesangial lupus nephritis (class I)
* Minimal mesangial lupus nephritis (class I)
* Mesangial proliferative lupus nephritis (class II)
* Mesangial proliferative lupus nephritis (class II)
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|Minimal mesangial lupus nephritis (class I)
|Minimal mesangial lupus nephritis (class I)
|
|
* The earliest and mildest form of 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)
|Mesangial proliferative lupus nephritis (class II)
|
|
* Microscopic hematuria and/or proteinuria
* Microscopic [[hematuria]] and/or [[proteinuria]]
|-
|-
|Focal lupus nephritis (class III)
|Focal lupus nephritis (class III)
|
|
* Hematuria and proteinuria
* [[Hematuria]] and [[proteinuria]]
* May also have hypertension, a decreased 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)
|Diffuse lupus nephritis (class IV)
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* Most common and most severe form
* Most common and most severe form


* 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 disease 7231154
* [[Hypocomplementemia]] (especially C3) and elevated anti-DNA levels, especially during active disease 7231154
|-
|-
|Lupus membranous nephropathy (class V) 
|Lupus membranous [[nephropathy]] (class V) 
|
|
* Hematuria and proteinuria
* [[Hematuria]] and [[proteinuria]]
* Nephrotic syndrome, hypertension, and reduced glomerular filtration rate
* [[Nephrotic syndrome]], [[hypertension]], and reduced [[glomerular filtration rate]]
* Hypocomplementemia
* [[Hypocomplementemia]]
|-
|-
|Advanced sclerosing lupus nephritis (class VI)
|Advanced sclerosing lupus nephritis (class VI)
|
|
* Slowly progressive renal dysfunction
* Slowly progressive [[renal dysfunction]]


* Proteinuria
* [[Proteinuria]]
|}
|}


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*[[Lupus nephritis]], an inflammation of the [[kidney]]s caused by SLE
*[[Lupus nephritis]], an inflammation of the [[kidney]]s caused by SLE
*Complement deficiency syndromes
*Complement deficiency syndromes


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Revision as of 13:38, 30 June 2017

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

Overview

Lupus may be classified into several subtypes according to clinical features which include: 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 glomerulonephritis and dermatologic manifestation. 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

Lupus may be classified into several subtypes according to clinical features which include:

  • Systemic lupus erythematosus (SLE)
  • Cutaneous lupus erythematosus (CLE)
  • Drug-induced lupus
  • Neonatal lupus

Based on this classification, SLE is a subtype of lupus that can be classified into different subclasses as well. CLE can be presented with or without systemic symptoms of the disease.

Systemic lupus erythematosus (SLE) may be classified into several subtypes based on glomerulonephritis and dermatologic manifestation.

SLE classification based on dermatologic manifestations

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)
  • Intermittent cutaneous lupus erythematosus (ICLE)
Subtypes Manifestation/subclass
Acute cutaneous lupus erythematosus (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

SLE classification based on glomerulonephritis

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)
  • Advanced sclerosing lupus nephritis (class VI)
Subtype Manifestation
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)
    • Chilblain lupus erythematosus (Hutchinson)
    • Lupus erythematosus-lichen planus overlap syndrome
    • Lupus erythematosus panniculitis (Lupus erythematosus profundus)
    • Subacute cutaneous lupus erythematosus, which causes nonscarring skin lesions on patches of skin exposed to sunlight.[1]
    • Tumid lupus erythematosus
    • Verrucous lupus erythematosus (Hypertrophic lupus erythematosus)
  • 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.[1]
  • Childhood systemic lupus erythematosus, the pediatric variant of systemic lupus erythematosus.
  • Drug-induced lupus erythematosus, a drug-induced form of SLE; this type of lupus can occur equally in either gender.
  • Lupus nephritis, an inflammation of the kidneys caused by SLE
  • Complement deficiency syndromes
Trunk
Extremity
Lupus Erythematosus Chronicus Verrrucous
Extremity


Trunk
Lupus Erythematosus Profundus
Extremity
Subacute Cutaneous Lupus Erythematosus
Face
Head
Trunk
Systemic Lupus Erythematosus
Face

References

  1. ↑ 1.0 1.1 "Handout on Health: Systemic Lupus Erythematosus". The National Institute of Arthritis and Musculoskeletal and Skin Diseases. National Institutes of Health. 2003. Retrieved 2007-11-23. Unknown parameter |month= ignored (help)
  2. ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2.16 2.17 2.18 2.19 2.20 2.21 2.22 2.23 2.24 2.25 2.26 2.27 2.28 2.29 2.30 2.31 2.32 "Dermatology Atlas".

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