The Concept of Early Systemic Sclerosis Following 2013 ACR\EULAR Criteria for the Classification of Systemic Sclerosis

For many years observational studies and clinical trials on systemic sclerosis (SSc) have been carried out only on patients who met the 1980 American College of Rheumatology-ACR preliminary classification criteria. However, this lead to the exclusion from all those studies of subset patients, particularly those with a limited cutaneous SSc-sine scleroderma subset, because, despite a diagnosis of SSc based on Raynaud’s phenomenon (RP) associated with digital pitting scars/ulcers, or by sclerodactyly and telangiectasia and/or typical esophagopathy and/or interstitial fibrosis detected by High Resolution Computed Tomography of the lungs, they did not satisfy the classification criteria. In that setting, LeRoy and Medsger proposed to label as affected by limited SSc (lSSc) or early SSc, cases presenting with RP associated with an SSc-type nailfold capillary pattern and/or SSc-selective autoantibodies. In 2008, Koenig et al. validated these criteria and proposed to name early SSc, or pre-scleroderma, patients with RP and either a scleroderma marker autoantibody or typical capillaroscopy abnormalities or both, who had been clearly shown to have high probabilities but not the certainty to develop definite SSc during a 20-year follow-up. This definition has been recently challenged by the development of the new ACR/EULAR criteria for the classification of SSc. At present, to be labeled as affected by early SSc, or pre-scleroderma, a patient should suffer from RP associated with either scleroderma marker autoantibodies or typical capillaroscopy findings and should not meet the 2013 ACR/EULAR criteria for the classification of SSc nor should he/she meet the criteria for SSc sine scleroderma.