Systemic Lupus Erythematosus ( SLE ) - Classification Criteria .pdf

This presentation describes the EULAR / ACR classification criteria for SLE.

Remember , classification criteria is not the same as diagnostic criteria.

Classification criteria is mainly used to select patients for clinical trials.

1. CLASSIFICATION CRITERIA FOR SLE

2. SYSTEMIC LUPUS ERYTHEMATOSUS
Systemic lupus erythematosus (SLE) is a complex autoimmune
disease with variable clinical features.

SLE manifestations are associated with multiple autoantibodies,
ensuing immune complex formation and deposition, and other
immune processes.

3. The classification criteria
previously used was the

Systemic ( S)
Lupus ( L )
International ( I )
Collaborating ( C )
Clinic (C )

Criteria ( 2012 )

4. European League Against Rheumatism/
American College of Rheumatology
classification criteria for
systemic lupus erythematosus
( 2019 )
The present criteria is the

5. Entry Criterion

[ Testing by immunofluorescence on HEp-2 cells or a solid phase ANA
screening immunoassay with at least equivalent performance is highly
recommended ]
Anti nuclear antibodies ( ANA ) at a titre of more than or equal to
1:80 on HEp 2 cells or an equivalent positive test ( ever )

6. If the entry criteria is absent , do not classify as SLE.

If present , apply additive criteria.
Additive criteria includes

7 clinical domains
and
3 immunology domains

7. Clinical Domains - 7
●Constitutional
●Hematologic
●Neuropsychiatric
●Mucocutaneous
●Serosal
●Musculoskeletal
●Renal

8. Immunology Domains - 3
●Antiphosphipid antibodies
●Complement proteins
●SLE specific antibodies

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

10. Clinical domains and criteria

11. Constitutional
Fever - 2 points

Fever - Temperature > 38.3 °C

12. Hematologic
Leucopenia - 3 points
Thrombocytopenia - 4 points
Autoimmune hemolysis - 4 points

Leucopenia - WBC count < 4000 cells / mm3
Thrombocytopenia - Platelet count < 100,000 cells / mm3
Autoimmune hemolysis - Evidence of hemolysis such as reticulocytosis , low
haptoglobin , elevated indirect bilirubin , elevated LDH and positive Direct
Coombs Test

13. Neuropsychiatric
Delirium - 2 points
Psychosis - 3 points
Seizure - 5 points

Psychosis - Characterised by
(1) delusions and/or hallucinations without insight and
(2) absence of delirium
Seizure - Primary generalised seizure or partial / focal seizure

14. Delirium -
Characterised by
(1) change in consciousness or level of arousal with reduced ability to focus,
(2) symptom development over hours to <2 days,
(3) symptom fluctuation throughout the day,
(4) either
(a) acute/subacute change in cognition (eg, memory deficit or disorientation),
or
(b) change in behaviour, mood, or affect (eg, restlessness, reversal of sleep/wake
cycle)

15. Mucocutaneous
Non scarring alopecia - 2
Oral ulcers - 2
Subacute cutaneous or discoid lupus - 4
Acute cutaneous lupus - 6

Non-scarring alopecia observed by a clinician*
Oral ulcers observed by a clinician*

16. Subacute cutaneous lupus erythematosus observed by a clinician*:
Annular or papulosquamous (psoriasiform) cutaneous eruption, usually
photodistributed

Discoid lupus erythematosus observed by a clinician*:
Erythematous-violaceous cutaneous lesions with secondary changes of
atrophic scarring, dyspigmentation, often follicular
hyperkeratosis/haematological(scalp), leading to scarring alopecia on the
scalp

17. If skin biopsy is performed, typical changes must be present.
Subacute cutaneous lupus: interface vacuolar dermatitis consisting of a
perivascular lymphohistiocytic infiltrate, often with dermal mucin noted.
Discoid lupus: interface vacuolar dermatitis consisting of a perivascular
and/or periappendageal lymphohistiocytic infiltrate. In the scalp, follicular
keratin plugs may be seen. In longstanding lesions, mucin deposition and
basement membrane thickening may be noted

18. Acute Cutaneous Lupus :
Malar rash or generalised maculopapular rash observed by a clinician
If skin biopsy is performed, typical changes must be present: interface
vacuolar dermatitis consisting of a perivascular lymphohistiocytic infiltrate,
often with dermal mucin noted. Perivascular neutrophilic infiltrate may be
present early in the course.

19. Serosal
Pleural or Pericardial effusion - 5 points
Acute pericarditis - 6

Pleural or pericardial effusion - Imaging evidence (such as ultrasound,
X-ray, CT scan, MRI) of pleural or pericardial effusion, or both

20. Acute Pericarditis - Two or more of
(1) pericardial chest pain (typically sharp, worse with inspiration,
improved by leaning forward),
(2) pericardial rub,
(3) electrocardiogram (EKG) with new widespread ST-elevation or
PR depression
(4) new or worsened pericardial effusion on imaging (such as
ultrasound, X-ray, CT scan, MRI

21. Musculoskeletal
Joint involvement - 6 points

Joint EITHER
(1) synovitis involving two or more joints characterised by swelling or
effusion OR
(2) tenderness in two or more joints and at least 30 min of morning stiffnes

22. Renal
Proteinuria > 0.5 g / 24 hours - 4 points
Renal biopsy - Class II or V Lupus - 8 points
Renal biopsy - Class III or IV Lupus - 10 points

Proteinuria >0.5 g/24 hours by 24 hours urine or equivalent spot urine
protein-to-creatinine ratio

23. Class II: Mesangial proliferative lupus nephritis: purely mesangial
hypercellularity of any degree or mesangial matrix expansion by light
microscopy, with mesangial immune deposit. A few isolated subepithelial
or subendothelial deposits may be visible by immune-fluorescence or
electron microscopy, but not by light microscopy

24. Class III: Focal lupus nephritis: active or inactive focal, segmental or
global endocapillary or extracapillary glomerulonephritis involving <50% of
all glomeruli, typically with focal subendothelial immune deposits, with or
without mesangial alterations

25. Class IV: Diffuse lupus nephritis: active or inactive diffuse, segmental or
global endocapillary or extracapillary glomerulonephritis involving ≥50% of
all glomeruli, typically with diffuse subendothelial immune deposits, with or
without mesangial alterations. This class includes cases with diffuse wire
loop deposits but with little or no glomerular proliferation

26. Class V : Membranous lupus nephritis: global or segmental subepithelial
immune deposits or their morphological sequelae by light microscopy and
by immunofluorescence or electron microscopy, with or without mesangial
alterations

27. Immunology domains and criteria

28. Anti phospholipid antibodies
Anti cardiolipin antibodies or
Anti beta 2 GP1 antibodies or
Lupus anticoagulant - 2 points

Anticardiolipin antibodies (IgA, IgG, or IgM) at medium or high titre (>40 A
phospholipids (APL), GPL or MPL units, or >the 99th percentile) or
positive anti-β2GP1 antibodies (IgA, IgG, or IgM) or
positive lupus anticoagulant

29. Complement proteins
Low C3 or Low C4 - 3 points
Low C3 and Low C4 - 4 points

C3 and / or C4 below the lower limit of normal

30. SLE specific antibodies
Anti ds DNA antibodies
or
Anti Smith antibodies - 6 points

Anti-dsDNA antibodies in an immunoassay with demonstrated ≥90% specificity for
SLE against relevant disease controls OR anti-Sm antibodies