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 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)	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 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)	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 7231154
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

- 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

Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]

Trunk

Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]

Extremity

Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]
Lupus erythematosus chronicus disseminatus superficialis. Adapted from Dermatology Atlas.^[2]