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: Raviteja Guddeti, M.B.B.S. [2] Kiran Singh, M.D. [3]
Overview
Lupus is a chronic autoimmune disease in which the body's own defense system attacks otherwise healthy tissue. Clinically, it can affect multiple organ systems including the heart, skin, joints, kidneys and nervous system.
Classification
- Systemic lupus erythematosus may be classified into several subtypes based on glomerulonephritis, dermatologic features, and [classification method 2], and [classification method 3].
- SLE may be classified according to dermatologic manifestations into 3 subtypes: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE)
- 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).
- SLE may be classified according to dermatologic manifestations into 3 subtypes: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE)
- SLE classification according to dermatologic manifestations:
- Acute cutaneous lupus erythematosus (ACLE)
- Subacute cutaneous lupus erythematosus (SCLE)
- Chronic cutaneous lupus erythematosus (CCLE)
- Intermittent cutaneous lupus erythematosus (ICLE) {| class="wikitable" ! !subtypes ! ! |- |Acute cutaneous lupus erythematosus (ACLE) |
- SLE classification according to dermatologic manifestations:
- Localized ACLE (ie, malar rash, butterfly rash)
- Generalized ACLE
- Toxic epidermal necrolysis-like ACLE | | |- |Subacute cutaneous lupus erythematosus (SCLE) |
- Annular SCLE
- Papulosquamous SCLE
- Drug-induced SCLE
- 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 | | |}
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 immune deposits that are identified by immunofluorescence alone or by both immunofluorescence and electron microscopy, but such patients do not have light microscopic abnormalities |
|
Mesangial proliferative lupus nephritis (class II) | microscopic hematuria and/or proteinuria
Light microscope: mesangial hypercellularity (of any degree) or mesangial matrix expansion A few isolated subepithelial or subendothelial deposits may be seen on immunofluorescence or electron microscopy |
|
Focal lupus nephritis (class III) | hematuria and proteinuria
may also have hypertension, a decreased glomerular filtration rate, and/or nephrotic syndrome Light microscopy: Active or inactive endocapillary or extracapillary segmental glomerulonephritis (Less than 50 percent of glomeruli are affected) Electron microscopy: immune deposits in the subendothelial space of the glomerular capillary and mesangium Glomeruli affected by fibrinoid necrosis and crescents Presence of tubulointerstitial or vascular abnormalities |
Class III (A)=focal proliferative lupus nephritis
class III disease with active lesions Class III (C)=focal sclerosing lupus nephritis chronic inactive lesions with scarring |
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 7231154 Light microscopy: more than 50 percent of glomeruli are affected that display endocapillary with or without extracapillary glomerulonephritis and Mesangial abnormalities Electron microscopy: subendothelial deposits, at least during the active phase and diffuse wire loop deposits, but with little or no glomerular proliferation |
This class is classified based on the tissue involvement to segmentally (S) or globally (G), and based on the inflammatory activity (or chronicity) of the lesions into 6 subclasses include:
Class IV-S (A) Class IV-G (A) Class IV-S (A/C) Class IV-G (A/C) Class IV-S (C) Class IV-G (C) |
Systemic lupus erythematosus, or SLE, is the most common form of lupus.
Discoid lupus erythematosus causes a skin rash that doesn't go away.
Subacute cutaneous lupus erythematosus causes skin sores on areas of the body exposed to the sun.
Neonatal lupus affects newborns.
Drug-induced lupus can be caused by certain medicines.
There are several types of lupus; in general, when the word lupus alone is used, reference is to systemic lupus erythematosus. different manifestion Other types include:[1]
- Chronic cutaneous lupus erythematosus
- 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.[2]
- Localized discoid lupus erythematosus
- Generalized discoid lupus erythematosus
- Childhood discoid lupus erythematosus
- 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.[3]
- Tumid lupus erythematosus
- Verrucous lupus erythematosus (Hypertrophic lupus erythematosus)
- 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.[2]
- 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.[3]
- 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
Diagnosis
Physical Examination
Skin
Lupus Erythematosus Chronicus Disseminatus Superficialis
Face
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
-
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
Trunk
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
Extremity
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.[4]
Lupus Erythematosus Chronicus Verrrucous
Extremity
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
Trunk
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus chronicus verrucous. Adapted from Dermatology Atlas.[4]
Lupus Erythematosus Profundus
Extremity
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Lupus erythematosus profundus. Adapted from Dermatology Atlas.[4]
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Lupus erythematosus profundus. Adapted from Dermatology Atlas.[4]
Subacute Cutaneous Lupus Erythematosus
Face
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Subacute cutaneous lupus erythematosus. Adapted from Dermatology Atlas.[4]
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Subacute cutaneous lupus erythematosus. Adapted from Dermatology Atlas.[4]
-
Subacute cutaneous lupus erythematosus. Adapted from Dermatology Atlas.[4]
-
Subacute cutaneous lupus erythematosus. Adapted from Dermatology Atlas.[4]
Head
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Subacute cutaneous lupus erythematosus. Adapted from Dermatology Atlas.[4]
Trunk
-
Subacute cutaneous lupus erythematosus. Adapted from Dermatology Atlas.[4]
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Subacute cutaneous lupus erythematosus. Adapted from Dermatology Atlas.[4]
Systemic Lupus Erythematosus
Face
-
Systemic lupus erythematosus. Adapted from Dermatology Atlas.[4]
-
Systemic lupus erythematosus. Adapted from Dermatology Atlas.[4]
-
Systemic lupus erythematosus. Adapted from Dermatology Atlas.[4]
-
Systemic lupus erythematosus. Adapted from Dermatology Atlas.[4]
-
Systemic lupus erythematosus. Adapted from Dermatology Atlas.[4]
References
- ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Chapter 8. ISBN 0721629210.
- ↑ Discoid Lupus Erythematosus
- ↑ 3.0 3.1 "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) - ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 4.13 4.14 4.15 4.16 4.17 4.18 4.19 4.20 4.21 4.22 4.23 4.24 4.25 4.26 4.27 4.28 4.29 4.30 4.31 4.32 "Dermatology Atlas".