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

Abstract

OBJECTIVE: To examine the difference in clinical signs of peripheral vasculopathy in patients (pts) with limited (lcSSc) and diffuse cutaneus systemic sclerosis (dcSSc). PATIENTS AND METHODS: 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. RESULTS: 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). CONCLUSION: 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.