Evolution of The Classification Criteria for

Systemic Lupus Erythematosus

Edy Novery

World Lupus Day 1

World Lupus Day 2
Outline
Background ACR 1997

Definition/Epidemiology SLICC 2012

Long Journey of the

EULAR/ACR 2019
Classification Criteria

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They are in colours

But, They are one

entity: Umbrella

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 A LITTLE BIT OF HISTORY

Lupus is the latin word for ‘wolf’ Erythematosus means ‘red rashes’

In 1851, Dr. Cazenave discovered red rashes In 1885, Sir William Osler, Lupus not only the
on a patient’s face that looked like wolf bites. skin but many other organs or systems. The
The named ‘Discoid lupus erythematosus’. named ‘ Systemic Lupus Erythematosus’.

Bertias G, et al. Lupus. 2012;76 – 505.

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 EULAR/ACR
Background SLICC
Epidemiology LongSLICC
Journey ACR 1997
SLICC SLICC
SLICC 2012 SLICC
2019

Systemic Lupus Erythematosus (SLE)

Chronic auto-
immune disease
▪ Severity
▪ Disease
Various clinical Atypical Activity
manifestation manifestation
▪ Organ Damage

Brunner HI, et al. Arthritis

Rheum. 2008;58:556–62.

Early onset? Childhood vs Adult

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Bundhun PK, et al. Medicine
6
(Baltimore). 2017;96:e8086.
Delay in Diagnosis?

Barrier contribution:

1. Missdiagnosis
2. Knowledge gaps

Diagnosis ?
Management ?
Delay in 3 – 12 months
Prognosis ?
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Tamar BR, et al. Arthritis Care Res. 2018;70:420–7.
Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

Why we need ‘classification criteria’?

Early vs Late
ACR 1982 → 1997
Atypical manifestation
Optimal management SLICC 2012
Organ Damage
Prognosis
Diagnosis ?
Sensitivity dan Specificity?
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 Organ
involvement :
Renal
Mortality Neurology
L
A Hematology
Morbidity
T Disease
E Activity
Lupus in children at initial diagnosis: Brunner HI, et al. Arthritis Rheum. 2008;58(2):556–62.
30%–40% : Moderate-Severe Bundhun PK. Medicine (Baltimore). 2017;96(37):e8086.

Since 1997, the number of lupus patients has been

increasing → Mortality decreased
Thailand

Lack of data
Indonesia
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Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

Classification Criteria

ACR 1997

SLICC 2012
Clinical Imunology
New criteria?
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Criteria Classification is
not a similar with
Criteria diagnosis

EULAR
A clinical diagnosis has to be
established by Physian
(Rheumatologist)
❖ It includes many more aspects
than can be include formal criteria
❖ Formal classification criteria might
be a guide to establish clinical Citeria classification → These are helpful in defining homogenous treatment
diagnosis populationWorld
forLupus
studyDaypurposes EULAR 12
Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

Proportion : 20% of Lupus Patients

Ratio Female : Male = 9-10 : 1 World Lupus Day

Barsalou J, et al. Cur Opin Rheumatol 2013;25:616–22.
Weiss JE. Pediatr Rev 2012;33:62–73. 13
Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

6-18 per
100.000

20-30 per 6,3- 19,3 per

100.000 100.000

20-30 per No Data

100.000 20-30 per
>>> 100.000

>>

>
World Lupus Day Pineles D. Lupus. 2011;20:1182–7.
14
Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

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 Definition/
Background Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019
Epidemiology

Systemic Lupus Erythematosus

• Prototypic multisystem autoimmune disorder → chronic inflamation,
vasculitis, deposisi kompleks imun, dan Vaskulopati
• Atypical manifestation → episodic
• Multisystem
• Episodic→ Remission period
• Antinuclear antibody

Barsalou J, et al. Cur Opin Rheumatol 2013;25:616–22.

World Lupus Day Weiss JE. Pediatr Rev 2012;33:62–73. 16
 Natural History
SLE

‘Auto-antibody mediated
disease’

Natural History
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Bertsias GK, et al. Ann Rheum Dis 2010;69:1603–11.
 PATOGENESIS

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Mok CC. J Clin Pathol. 2003;56(7):481–90.
 2168
Childhood
120–140 SLE
patients

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Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

Guilermo B, et al. Nature Rev. 2018;18:79-93. World Lupus Day 21

 Long Journey of
Classification Criteria of SLE

Preliminary Proposed
1982 Revised ACR 2012 2019
criteria new criteria

Proposed EULAR/ACR
1971 1997 SLICC
modifications 2017 2019

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 1 ACR 1997 Any 4 of 11

M Any 4 of 17 (at least one immunological)

E 2 SLICC 2012 Histology→ Lupus Nephritis and ANA or
anti-dsDNA
E
T
ANA is entry criterion
3 EULAR/ACR 2019 10 Points weighted items
(highest in each domain counted only)

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1971

1982

1997
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Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

Any 4 of 11
• Nine items
Without clinical
weighting manifestation
certain criteria • Two items
immunological
manifestation
Clinical =
immunological

World Lupus Day Weiss JE. Pediatr Rev 2012;33:62–73. 25

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E
U
A 2
L
C 0
A
R 1
R
9
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Sensitivity

>95%

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 False positive ANA

ANA Positive
About 10-12.6% healthy children presented a positive
ANA test; there was no difference between genders, and
we observed a trend for higher frequency of ANA ≥ 1/80
among children between 5 years and 10 years.

Hilario MOE, et al. Clin Pediatr. 2004;43:637-42.

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 Two
Studies in
Children
Research

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 Penelitian Pioneer
New Criteria
(EULAR/ACR) 2019
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 Comparison: ACR 1997 vs SLICC 2012 vs
EULAR/ACR 2019
Perbedaan ACR 1997 SLICC 2012 EULAR/ACR 2019
Tahun Publikasi 1971, kemudian direvisi tahun 2012 2019
1982 dan 1997
Domain Klinis 9 kriteria 11 kriteria 7 Domain klinis

Domain Imunologis 2 kriteria 6 kriteria 3 Domain dan terbagi dalam

bobot kriteria
Sistem bobot Tidak Tidak Ya

Kriteria Khusus nefritis lupus Tidak Ya Ya, dalam perhitungan bobot

Syarat kriteria klinis atau Tidak ada syarat Ada, minimal satu kriteria klinis Ada, minimal 1 kriteria klinis
imunologis dan satu kriteria imunologis
Hasil pemeriksaan ANA Tidak wajib ada Tidak wajib ada Wajib, sebagai kriteria masuk

Gejala konstitusional Tidak ada Tidak ada Ada, keluhan demam

Syarat terpenuhi kriteria Minimal 4 kriteria Minimal 4 kriteria, dan terpenuhi Satu kriteria klinis dan total skor
satu kriteria klinis dan satu 10 poin serta kriteria masuk
kriteria imunologis terpenuhi
Sensitivitas 77-91% 92-99% 96,2%

Spesifitas 91-96% 74-88%

World Lupus Day 94,43% 34
Background Epidemiology Long Journey ACR 1997 SLICC 2012 Validitas

SLICC

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Background Epidemiology Long Journey ACR 1997 SLICC 2012 Validitas

Fonseca AR, et al. Adv Rheumatol. 2019;59:20–9.

World Lupus Day Faezi ST, et al. Int J Rheum Dis. 2014;17:394–99.
36
Petri M, et al. Lupus. 2004;13:829–37.
Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

Study Country Sensitivity (%) Spesificity (%)

Jialin T China
A
D ACR 1997 75,4 96,0
U SLICC 2012 92,0 84,0
L
T ACR/EULAR 2019 96,5 90,3
H Michelle P Multi-center
O
O ACR 1997 83 96
D SLICC 2012 97 84
ACR/EULAR 2019 91 89
World Lupus Day Petri M, et al. Arthritis Care Res. 2020;24:242–57. 37
Teng J,et al. Lupus. 2020;29:617–24.
Background Epidemiology Long Journey ACR 1997 SLICC 2012 EULAR/ACR 2019

Study Country Sensitivity (%) Spesificity (%)

C Fonsesca AR21 Brazil
H
ACR 1997 70,5 83,2
I
SLICC 2012 82,3 80,9
L
D EULAR/ACR 2019 87,7 67,4
H Batu ED34 Turki
O ACR 1997 68,7 94,8
O SLICC 2012 95,4 89,7
D
EULAR/ACR 2019 91,6 88,5

Adriana RF, et al. Adv Rheumatol. 2019;59:20–9.

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Which one is better?
ACR 1997 SLICC 2012 ACR/EULAR 2019

Validity ?

Sensitifity-specificity ?

Clinical
manifestation?
?
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 Take Home Massages

1. SLE is various manifestation 2. SLE is an auto-antibody mediated

and atypical manifestation disease

Three criteria classification: Early phase of SLE is crucial point

3. ACR 1997, SLICC 2012, and 4. to manage further management
EULAR/ACR 2019 and good outcome

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Thank You

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