1. ACR – EULAR Classification Criteria for Systemic Sclerosis May 25, 2013 Sindhu Johnson MD PhD

2. 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

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

4. 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

8. SSc diagnosis SSc classification

9. 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

10. 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

11. Classification schemes – Overview 1980199020002010 1980 – American College of Rheumatology 1988 – LeRoy: lcSSc and dcSSc 2001 – LeRoy: lSSc

12. 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%

13. Collaboration between experts and clinical epidemiologists Evaluate psychometric properties of candidate criteria Balance of expert based and data driven methods Avoid circularity of reasoning

14. Classification schemes – Overview Current: ACR-EULAR Collaboration 1980199020002010 1980 – American College of Rheumatology 1988 – LeRoy: lcSSc and dcSSc 2001 – LeRoy: lSSc

15. 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 Method Data-Driven Method Evaluate operating characteristics of SSc criteria Item reduction, weighting & scaling

16. Entry Criterion ? meets “Threshold” Multi-Criteria Decision Analysis Absolute Criteria Exclusion Criteria NO Not Systemic Sclerosis Systemic Sclerosis YES NO YES

18. 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)

19. 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