These findings suggest that COL22A1 levels are elevated in fibroblasts from patients with SSc-associated dermal fibrosis, and due to its role in mediating TGFβ regulation of ACTA2 and myofibroblast differentiation, COL22A1 may be involved in the pathogenesis of SSc dermal fibrosis. This evidence concerns the gene COL22A1 and systemic sclerosis.