ACR/EULAR Add Systemic Sclerosis Criteria

van den Hoogen F, Khanna D, Fransen F, et al. 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative,Arthritis & Rheumatism (2013) doi: 1002/art.38098 First published online: October 3, 2013

New classification criteria for systemic sclerosis (SSc), jointly drafted by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR), are not only more sensitive than older ACR criteria but will also enable faster and more accurate diagnosis of scleroderma, a collaborative study finds.

“Criteria that are more specific will allow for earlier identification and better treatment for those with systemic sclerosis,” says a statement by Janet Pope, MD, from Western University and St. Joseph’s Health Care London in Ontario, Canada, a leader of the joint ACR/EULAR committee.

Validation testing of the new classification criteria finds that skin thickening of the fingers (extending proximal to the metacarpophalangeal joints) alone is sufficient for a patient to be classified as having scleroderma.

If that single key sign is absent, seven other factors should be assessed to make a classification of scleroderma, according to the criteria:
•    General skin thickening of the fingers
•    Raynaud’s phenomenon
•    Fingertip lesions
•    Telangiectasia
•    Abnormal nailfold capillaries
•    Interstitial lung disease or pulmonary arterial hypertension
•    The presence of SSc-related autoantibodies

The new ACR/EULAR criteria were tested for specificity and sensitivity by assessing 200 cases of SSc and SSc-like disorders, and validated by experts comparing cases with scleroderma (n=268) and scleroderma-like disorders (n=137).

The validation testing finds the new SSc criteria 90% more sensitive and specific compared with a 75% sensitivity/specificity for the 1980 ACR criteria.

Use of the 1980 criteria resulted in around 20% of patients with limited cutaneous criteria being excluded from clinical studies, the joint study notes.