ACR * EULAR Classification Criteria for Systemic Sclerosis

Report
ACR – EULAR Classification Criteria for
Systemic Sclerosis
Sindhu Johnson MD PhD
May 25, 2013
Learning Objectives
• Highlight the limitations of pre-existing
classification criteria
• To discuss the development of the new
classification criteria
• To discuss the application of the 2013 classification
criteria
2
Disclosures
• American College of Rheumatology Classification
and Response Criteria Subcommittee of the
Committee on Quality Measures.
Co-convenors
Janet Pope
Frank van den Hoogen
Members
Jaap Fransen
Sindhu Johnson
Alan Tyndall
Murray Baron
Marco Matucci-Cerinic
Dinesh Khanna
Collaborators
Ray Naden
Christopher Denton
Oliver Distler
Armando Gabrielli
Jaap van Laar
Maureen Mayes
Virginia Steen
James Seibold
Phillip Clements
Thomas Medsger
Gabriela Riemekasten
Patricia Carreira
Collaborators
Lorinda Chung
Barri Fessler
Peter Merkel
Richard Silver
John Varga
Yannick Allanore
Ulf Mueller-Ladner
Madelon Vonk
Ulrich Walker
Susanna Cappelli
SSc
diagnosis
SSc
classification
ACR Preliminary Classification Criteria
for Systemic Sclerosis
Major criterion or 2 minor criteria
Major criterion
* Proximal scleroderma
Minor criteria
* Sclerodactyly
* Digital pitting or scars or loss of substance
from finger pad
* Bibasilar pulmonary fibrosis
Arthritis Rheum 1980; 23:581-90
ACR Preliminary Classification Criteria
for Systemic Sclerosis
1 major criterion or ≥ 2 minor criteria
Sensitivity 97% of definite SSc cases
Specificity 98% the comparison patients
Arthritis Rheum 1980; 23:581-90
Classification schemes – Overview
1980 – American College of Rheumatology
1988 – LeRoy: lcSSc and dcSSc
2001 – LeRoy: lSSc
1980
1990
2000
2010
Limitations of ACR classification criteria
for SSc
Miss subsets
New knowledge
Limited cutaneous SSc
Nailfold capillaries
Early SSc
SSc- specific antibodies
20%
SSc
diagnosis
SSc
classification
Collaboration between experts and clinical epidemiologists
Balance of expert based and data driven methods
Avoid circularity of reasoning
Evaluate psychometric properties of candidate criteria
Classification schemes – Overview
1980 – American College of Rheumatology
1988 – LeRoy: lcSSc and dcSSc
2001 – LeRoy: lSSc
1980
1990
2000
2010
Current:
ACR-EULAR
Collaboration
Item Reduction
(Delphi panel followed by
Nominal Group Technique) N = 23
Consensus Method
Item Generation
(Delphi panel) N = 168
Evaluate validity of items in SSc
and SSc mimickers
Collect prospective data in SSc
and SSc mimickers
Evaluate operating characteristics
of SSc criteria
Data-Driven Method
Item reduction, weighting &
scaling
Systemic Sclerosis
YES
Entry
Criterion
NO
Exclusion
Criteria
Absolute
Criteria
YES
Multi-Criteria
Decision
Analysis
? meets
“Threshold”
YES
NO
Not Systemic Sclerosis
Derivation sample
Validation sample
Validation sample
(N=200)
(N=405)
≤ 3 years disease
duration
(N=100)
1980 ARA SSc
Criteria
2001 LeRoy and
Medsger criteria
2013 ACR-EULAR
SSc Classification
Criteria
Sensitivity
Specificity
Sensitivity
Specificity
Sensitivity
Specificity
(95% CI)
(95% CI)
(95% CI)
(95% CI)
(95% CI)
(95% CI)
0.80
0.77
0.75
0.72
0.75
0.72
(0.72,
0.87)
(0.68,
0.84)
(0.70,
0.80)
(0.64,
0.79)
(0.70,
0.80)
(0.63,
0.79)
0.76
0.69
0.75
0.78
0.80
0.76
(0.68, 0.84) (0.68, 0.84) (0.70, 0.80) (0.70, 0.85) (0.69, 0.88) (0.53, 0.92)
0.95
0.93
0.91
0.92
0.91
0.90
(0.90,
0.98)
(0.86,
0.97)
(0.87,
0.94)
(0.86,
0.96)
(0.83,
0.96)
(0.70,
0.99)
Learning Objectives
• Highlight the limitations of pre-existing
classification criteria
• To discuss the development of the new
classification criteria
• To discuss the application of the 2013 classification
criteria
19

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