ACR – EULAR Classification Criteria for Systemic Sclerosis

May 25, 2013

Sindhu Johnson MD PhD

• 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

Learning Objectives

• American College of Rheumatology Classification and Response Criteria Subcommittee of the Committee on Quality Measures.

Disclosures

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 classificatio

n

Major criterion * Proximal scleroderma

Minor criteria * Sclerodactyly * Digital pitting or scars or loss of substance from finger pad * Bibasilar pulmonary fibrosis

Major criterion or 2 minor criteria

ACR Preliminary Classification Criteria for Systemic Sclerosis

Arthritis Rheum 1980; 23:581-90

Sensitivity 97% of definite SSc cases

Specificity 98% the comparison patients

1 major criterion or ≥ 2 minor criteria

ACR Preliminary Classification Criteria for Systemic Sclerosis

Arthritis Rheum 1980; 23:581-90

Classification schemes – Overview

1980 1990 2000 2010

1980 – American College of Rheumatology

1988 – LeRoy: lcSSc and dcSSc

2001 – LeRoy: lSSc

Limited cutaneous SSc

Early SSc

New knowledge

Nailfold capillaries

SSc- specific antibodies

Limitations of ACR classification criteria for SSc

Miss subsets

SSc diagnosis

SSc classification

20%

Collaboration between experts and clinical epidemiologists

Evaluate psychometric properties of candidate criteria

Balance of expert based and data driven methods

Avoid circularity of reasoning

Classification schemes – Overview

Current:ACR-EULAR Collaboration

1980 1990 2000 2010

1980 – American College of Rheumatology

1988 – LeRoy: lcSSc and dcSSc

2001 – LeRoy: lSSc

Item Generation(Delphi panel) N = 168

Item Reduction(Delphi panel followed by

Nominal Group Technique) N = 23

Evaluate validity of items in SSc and SSc mimickers

Collect prospective data in SSc and SSc mimickers

Consensus M

ethodD

ata-Driven M

ethod

Evaluate operating characteristics of SSc criteria

Item reduction, weighting & scaling

EntryCriterion

? meets “Threshold”

Multi-CriteriaDecisionAnalysis

AbsoluteCriteria

ExclusionCriteria

NO

Not Systemic Sclerosis

Systemic Sclerosis

YES

YES

NO

YES

Derivation sample

(N=200)

Validation sample

(N=405)

Validation sample

≤ 3 years disease duration

(N=100)

Sensitivity

(95% CI)

Specificity

(95% CI)

Sensitivity

(95% CI)

Specificity

(95% CI)

Sensitivity

(95% CI)

Specificity

(95% CI)

1980 ARA SSc Criteria

0.80

(0.72, 0.87)

0.77

(0.68, 0.84)

0.75

(0.70, 0.80)

0.72

(0.64, 0.79)

0.75

(0.70, 0.80)

0.72

(0.63, 0.79)

2001 LeRoy and Medsger criteria

0.76

(0.68, 0.84)

0.69

(0.68, 0.84)

0.75

(0.70, 0.80)

0.78

(0.70, 0.85)

0.80

(0.69, 0.88)

0.76

(0.53, 0.92)

2013 ACR-EULAR SSc Classification Criteria

0.95

(0.90, 0.98)

0.93

(0.86, 0.97)

0.91

(0.87, 0.94)

0.92

(0.86, 0.96)

0.91

(0.83, 0.96)

0.90

(0.70, 0.99)

• 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

Learning Objectives