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

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 may be classified into several subtypes based on glomerulonephritis and dermatologic features. 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)

subtypes
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
  • 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)

Lupus membranous nephropathy (class V)  diffuse thickening of the glomerular capillary wall on light microscopy

subepithelial immune deposits (either global or segmental involvement) on immunofluorescence or electron microscopy

mesengial involvement

Advanced sclerosing lupus nephritis (class VI) slowly progressive renal dysfunction

proteinuria

global sclerosis of more than 90 percent of glomeruli

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)
  • 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
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. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Chapter 8. ISBN 0721629210.
  2. ↑ Discoid Lupus Erythematosus
  3. ↑ 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. ↑ 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".

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