Peripheral vasculopathy in patients with systemic sclerosis: difference in limited and diffuse subset of disease

To examine the difference in clinical signs of peripheral vasculopathy in patients (pts) with limited (lcSSc) and diffuse cutaneus systemic sclerosis (dcSSc).

Ninety one patients with systemic sclerosis (39 with lcSSc and 52 with dcSSc) have been assessed for the presence of clinical signs of vascular injury: Raynaud's phenomenon, severity of capillary damage on capillaroscopy, presence or absence of finger-tip ulcers or pitting scars, presence of telangiectasias and radiographic signs of finger-tip osteolysis. Statistical significance of difference in clinical manifestations of peripheral vasculopathy in pts with lcSSc and dcSSc was assessed using the Mann-Whitney and X2-test.

Duration of Raynaud's phenomenon before manifestation of skin or internal organ damage, was significantly longer (z=-2.54, p=0.004) in patients with lcSSc (5.4 years) than in patients with dcSSc (1.9 years). Using the technique of nailfold capillaroscopy, we found normal capillaries or non-specific capillary change in 10.2% pts with lcSSc and only in 2.0% pts with dcSSc. Enlarged capillaries without significant loss of capillaries were found in 38.5% pts with lcSSc, and 11.5% pts with dcSSc (p=0.05). But severe capillary damage, with significant loss of capillaries, was noticed more frequently in pts with dcSSc (dcSSc/lcSSc: 86.5%/51.3%, p=0.002). Pitting scars or digital ulcers were found in 46.2% pts with lcSSc and 67.3% pts with dcSSc (p=0.04). We did not notice significant difference in frequency of finger-tip osteolysis (lcSSc/dcSSc: 23.1%/21.2%, p>0.05) and telangiectasias (lcSSc/dcSSc: 46.2%/53.8%, p>0.05).

Severe capillary damage and digital ulcers are more common in patients with diffuse cutaneus systemic sclerosis, but finger-tip osteolysis and telangiectasias are equally frequent in both form of disease.