EULAR/ACR New Classification Criteria for Systemic Lupus Erythematosus (SLE)

The new EULAR/ACR classification criteria use anti-nuclear antibodies (ANA) as an entry criterion. (Non-infectious) fever is the one new criterion. All criteria items now have individual weights (from 2 to 10) and are structured in domains, within which only the highest item is counted. There is one common attribution rule, counting criteria only if there is no more likely alternative explanation. Ten points are sufficient for classification. The new criteria have reached a sensitivity of 96.1% and a specificity of 93.4%.

Classification criteria for systemic lupus erythematosus.

Entry criterion
Anti-nuclear antibodies (ANA) at a titer of ≥1:80 on HEp-2 cells or an equivalent positive test (ever)

• if absent, do not classify as SLE
• If present, apply additive criteria

Additive criteria

• Do not count a criterion if there is a more likely explanation the SLE.
• Occurrence of a criterion on at least one occasion is sufficient.
• SLE classification requires at least one clinical criterion and ≥10 points.
• Criteria need not occur simultaneously.
• Within each domain, only the highest weighted criterion is counted toward the total score§.

Clinical domains and criteria	Weight	Immunology domains and criteria	Weight
Constitutional		Antiphospholipid antibodies
Fever	2	Anti-cardiolipin antibodies OR Anti-ß2GP1 antibodies OR Lupus anticoagulant	2
Hematologic		Complement proteins
Leukopenia Thrombocytopenia Autoimmune hemolysis	3 4 4	Low C3 OR low C4 Low C3 AND low C4	3 4
Neuropsychiatric		SLE-specific
Delirium Psychosis Seizure	2 3 5	Anti-dsDNA antibodies* OR Anti-Smith antibody	6
Mucocutaneous
Non-scarring alopecia Oral ulcers Subacute cutaneous OR discoid lupus Acute cutaneous lupus	2 2 4 6
Serosal
Pleural or pericardial effusion Acute pericarditis	5 6
Musculoskeletal
Joint involvement	6
Renal
Proteinuria >0.5 g/24 hs Renal biopsy Class II or V lupus nephritis Renal biopsy Class III or IV lupus nephritis	4 8 10
Total score:

Classify as Systemic Lupus Erythematosus with a score of 10 or more if entry criterion fulfilled.

§Additional criteria items within the same domain will not be counted.
*Note: In an assay with at least 90% specificity against relevant disease controls.

 

References:

1. Aringer M, Leuchten N, Johnson SR. New Criteria for Lupus. Curr Rheumatol Rep. 2020 May 13;22(6):18. [Medline]
2. Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, Smolen JS, Wofsy D, Boumpas DT, Kamen DL, Jayne D, Cervera R, Costedoat-Chalumeau N, Diamond B, Gladman DD, Hahn B, Hiepe F, Jacobsen S, Khanna D, Lerstrøm K, Massarotti E, McCune J, Ruiz-Irastorza G, Sanchez-Guerrero J, Schneider M, Urowitz M, Bertsias G, Hoyer BF, Leuchten N, Tani C, Tedeschi SK, Touma Z, Schmajuk G, Anic B, Assan F, Chan TM, Clarke AE, Crow MK, Czirják L, Doria A, Graninger W, Halda-Kiss B, Hasni S, Izmirly PM, Jung M, Kumánovics G, Mariette X, Padjen I, Pego-Reigosa JM, Romero-Diaz J, Rúa-Figueroa Fernández Í, Seror R, Stummvoll GH, Tanaka Y, Tektonidou MG, Vasconcelos C, Vital EM, Wallace DJ, Yavuz S, Meroni PL, Fritzler MJ, Naden R, Dörner T, Johnson SR. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis. 2019;78(9):1151–1159. [Medline]
3. Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019;71(9):1400–1412. [Medline]

 

Created Mar 06, 2021.