Lupus Erythematosus: Classification

Systemic lupus erythematosus (SLE): the American College of Rheumatology (ACR) classification criteria are increasingly being replaced by the classification presented below. This was modified in 2012 by the Systemic Lupus International Collaborating Clinics (SLICC) group based on the American College of Rheumatology (ACR) classification criteria . A prerequisite for establishing a diagnosis of SLE is that 4 criteria (at least 1 of which is clinical and 1 immunologic) are met or, in the case of positive ANA or anti-dsDNA antibodies, a histologic diagnosis of lupus nephritis (inflammation of the kidneys) is made. The SLICC criteria are not diagnostic criteria. Note: The SLICC criteria became less well known, although they had better sensitivity (92-97% vs. 77-90%) with poorer specificity (74-88 vs. 91-96%) compared to the 1982 classification criteria. Meanwhile, new classification criteria for lupus patients were presented at the annual international congress of the European League Against Rheumatism (EULAR) (see below). SLE Classification Criteria: Systemic Lupus International Collaborating Clinics (SLICC) Classification Criteria.

Clinical criteria
  • Acute cutaneous lupus erythematosus including butterfly erythema: fixed erythema (skin redness; flat or raised) on the face (nasal and cheek areas), usually excluding the nasolabial folds (“nasolabial furrow”)
  • Chronic cutaneous lupus erythematosus (e.g., localized or generalized discoid lupus erythematosus: erythematous, raised patches of skin with nonremovable keratotic (keratinizing) portions and follicular hyperkeratosis (excessive keratinization of the skin))
  • Oral (“belonging to the mouth”) and/or nasopharyngeal (“belonging to the nasopharynx”) ulcers
  • Serositis
    • Pleurisy (inflammation of the pleura) or pericardial pain lasting more than one day
    • Pericarditis (inflammation of the pericardium).
    • Peritonitis (peritonitis)
    • Ascites (abdominal dropsy)
  • Renal involvement: Single urine: protein/creatinine ratio (P/C ratio) or protein excretion (protein excretion) in 24 h pooled urine > 0.5 g
  • Neurologic involvement: e.g., epilepsy (seizures), psychosis, myelitis (inflammation of the spinal cord), neuropathy (diseases of the peripheral nerves)
  • Hemolytic anemia (anemia caused by increased or premature decay of erythrocytes/red blood cells (hemolysis)) with reticulocytosis
  • Leukocytopenia (< 4,000 leukocytes (white blood cells)/μl) or lymphocytopenia (< 1,500/μl).
  • Thrombocytopenia (< 100,000/μl/decreased platelet count).
Immunological criteria
  • ANA titers above the laboratory reference value.
  • Anti-dsDNA antibody
  • Anti-Sm antibody
  • Anti-phospholipid antibodies (anti-cardiolipin and an- ti- β 2-glycoprotein I [IgA, IgG, or IgM] antibodies; false-positive VDRL [Venereal Disease Research Laboratory] test).
  • Decreased complement (C3, C4, or CH50).
  • Direct Coombs test (without hemolytic anemia).

New EULAR/ACR classification criteria for lupus patients (presented at the annual international congress of the European League Against Rheumatism (EULAR)).

In an initial comparison of 1,000 SLE patients, the new EULAR/ACR criteria are reported to perform much better than the old ACR (85% and 95%), with sensitivity and specificity of 98% and 97%, respectively.

Clinical domains and criteria Weighting
constitutional symptoms fever 2
Skin Non-scarring alopeciaoral ulcerasubacute cutaneous or discoid LEacute cutaneous LD. 2246
Arthritis Synovitis ≥ 2 joints or tenderness in ≥ 2 joints with morning stiffness ≥ 30 minutes 6
Neurology DeliriumPsychosisSeizures 236
Serositis Pleural or pericardial effusionacute pericarditis 56
Hematology LeukopeniaThrombopeniaAutoimmune hemolysis 344
Kidneys Proteinuria > 0.5 g/24 hLupus nephritis (histol.) type II, VLupus nephritis (histol.) type III, IV 4810
Immunological domains and criteria Weighting
Antiphospholipid-Ak aCL > 40 GPL or aβ2GPI > 40 GPL or LA +. 2
Complement C3 or C4 diminishedC3 and C4 diminished 34
high specific auto ac Anti-dsDNS-AkAnti-Sm-Ak 66

Lupus nephritis classification

Class Description
1 Minimum mesangial LGN
2 Mesangioproliferative LGN
3 Focal LGN (<50% of glomeruli) active/sclerosed, segmental or global
4 Diffuse LGN (>50% of glomeruli) active/sclerosed, segmental or global
5 Membraneous LGN
6 Sclerosed LGN (>90% of glomeruli).

Legend: LGN: lupus glomerulonephritis.