OBJECTIVES: The aim was to define clinical characteristics and long-term survival of patients with dcSSc and positive ACA. METHODS: We identified all cases of ACA+ SSc in our cohort (n = 1313). Those with dcSSc (ACA+ diffuse) were compared with representative groups of consecutive ACA+ patients with limited subset (ACA+ limited) and ACA- dcSSc (non-ACA diffuse). RESULTS: Thirty-five patients (2.7%) were ACA+ di...