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: 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 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 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), 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:

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. Lee HJ, Sinha AA (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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