Distinctive clinical phenotype of anti-centromere antibody-positive diffuse systemic sclerosis

Abstract

ACA⁺ dcSSc is uncommon and has a distinct clinical phenotype, with a more insidious onset of skin and organ involvement. Even in dcSSc, ACA appears protective for organ-based complications, namely interstitial lung disease and scleroderma renal crisis, and is associated with a better survival than expected in dcSSc.