Macroangiopathy of the upper extremities in progressive systemic sclerosis

Raynaud's phenomenon is a hallmark of progressive systemic sclerosis (SSc). While nailfold capillary changes are well known, morphological changes in larger arteries have less been focused on.

In the study presented we examined how often in digital subtraction angiography organic changes of the arteries of the fingers, hands and forearms were observed, whether they showed a typical picture like the changes in nailfold capillaries and whether they correlated with cutaneous sclerosis, Raynaud's phenomenon and serological findings. 29 patients were examined, 14 with acroscleroderma and 15 with proximal ascending sclerosis.

In 27 of those 29 patients a stenosis of the arteries of the upper extremity was found. The frequency of arterial occlusions decreased from the fingers (26 patients) to the forearms (9 patients with occlusion of the ulnar artery, none with occlusion of the radial artery). The arteries of 47% of patients with proximal ascending sclerosis showed no reactivity towards the alpha-sympatholyticum tolazoline hydrochloride due to severe organic changes while in patients with acroscleroderma only 14% of patients did not respond to tolazoline hydrochloride. The severity of Raynaud's phenomenon did not correlate with the severity of the angiographic findings. In patients with stenoses refractory to tolazoline hydrochloride and in those with occlusions Scl-70 autoantibodies were more frequently positive than in other patients with SSc (44% compared to 23%).

As in SSc the severity of the organic arterial changes is in close correlation with the extent of the cutaneous sclerosis and with the serological findings the arterial system should gain much more importance in the diagnosis and therapy of SSc.