OBJECTIVE: To determine whether the type of skin involvement predicts the risk of developing restrictive lung disease (RLD) in patients with systemic sclerosis and their probability of survival. METHODS: Demographic data and clinical data of 2205 patients with systemic sclerosis over a 30-year period were retrospectively analyzed, and these patients were classified into four subtypes based on the type of skin fibrosis: type 0 (no skin involvement), type 1 (limited to the metacarpophalangeal joints), type 2 (distal to the elbow/knee), and type 3 (near the elbow/knee). Regression analysis was used to determine the clinical characteristics of the different subtypes, and the Kaplan-Meier method and the Cox proportional risk model were used to compare the time to development of RLD in the different subgroups and the survival rates between the different subgroups. RESULTS: The presence and severity of RLD were positively correlated with skin subtypes (P<0.001), and the prevalence of RLD increased progressively from 51.9% in type 0 to 76.7% in type 3 (P<0.001). Scleroderma type 2 exhibited a different cellular phenotype and moderate risk of developing RLD (higher than type I and lower than type 3, p<0.001) and a unique autoantibody profile than types 1 and 3. Patients with type 2 had a higher proportion of anti-adhesion antibodies than type 1 (28.9% vs 44.1%, p=0.001), whereas the presence of anti-topoisomerase I antibodies was similar to that of type 3. Anti-adnexin antibodies were a protective factor for the development of RLD (OR=0.33, p<0.001), while anti-topoisomerase I antibodies were a risk factor (OR=1.6, p=0.01). Mortality was also intermediate for type 2 relative to type 1 and type 3. CONCLUSION: These data suggest that the current classification of scleroderma into diffuse and limited types according to skin involvement allows for the misclassification of intermediate patient groups in between, with specific autoantibody profiles, disease processes, and clinical prognosis.