Abstract  Although digital ulceration frequently occurs in patients with systemic sclerosis, there have been few reports on macrovascular involvement. Macrovascular disease in systemic sclerosis has recently been described. We retrospectively reviewed the medical records and brachial angiographic findings of 19 systemic sclerosis patients, who exhibited Raynaud’s phenomenon and digital ulceration. We found that ulnar artery involvement is frequent in systemic sclerosis, although the precise mechanism is not known. There was no significant difference in risk factors of macrovascular disease between ulnar artery-involved patients and not-involved subjects. Thirteen patients underwent surgical intervention; five of the 13 patients had vascular graft performed due to ulnar artery involvement. We suggest that angiographic screening and early surgical intervention such as revascularization should be considered in patients with systemic sclerosis who manifest a severe form of Raynaud’s phenomenon and/or digital ulceration and especially in patients with diffuse sclerosis.