Background Systemic sclerosis (SSc) is a connective tissue disease characterized by excessive collagen deposition and by vascular hyperactivity and obliteration of microvasculopathy. Raynaud phenomenom appears years before other scleroderma patterns. The capillaroscopy findings and their changes could help us to define the diagnosis of scleroderma in early stages. The modified Scleroderma Health Assessment Questionnaire (SHAQ) helps to monitoring activity of disease.
Methods We evaluate 102 patients with systemic sclerosis, 81.4% patients with limited SSc (lSSc), 9.8% patients with CREST syndrome, 6.9% patients with diffuse SSc (dSSc), 1.9% patient with overlap syndrome with SSc.
We examinated: mRodnan score, organ involvement, including ECHO, CT of lung, capillaroscopy, evidence of autoantibodies - Scl70, anticentromere antibodies, questionnaire: HAQ-DI, SHAQ.
Defect of microvascullatory plays key role in pathogenesis of SSc. It appeared before development of skin sclerosis. Degree of capillaroscopy changes (dilatation of capillars, microhemorhagie, avascular places. Defect of organization, low number of capillary., microhemorhagie) could correlated with systemic microvasculopathy and involvement of visceral organs.
Results In observation group patients with dSSc had higher frequency of pulmonary hypertension (28.6% vs. 12.0% in lSSc patients and vs. 10% patient with CREST). Lung involvement control by diffuse capacity of CO (DLCO) was 85.7% in dSSc vs. to 66.2% in lSSc, although fibrosis detected by CT was similar. Activity score was higher in patients with dSSc (4.41), vs 2.33 in patients with lSSc, 2.5 in patient with CREST. Skin score mRodnan was higher than 14 in 87.5% in patients with dSSc, to 16.7% in patients with lSSc and 40% in patients with CREST. Visual analogue scales in presence of Raynaud phenomenon, pulmonary involvement, involvement of gastrointestinal tract, activity of SSc were higher in patients with dSSc. SSc HAQ score was a little bit higher in patients with dSSc (1,6) and CREST (1.67) to lSSc (1.43). Antinuclear antibodies (Hep2) were positive in 86.2% patients, autoantibodies to anti-DNA topoisomerase were in 33.8% and anti-centromere autoantibodies in 9.2% patients. In all patients with SSc we find picture of SSc in different stages. In 7 patients with episode of Raynaud phenomenon only during winter we found early SSc changes (in number of capillaries, dilatation, megacapillary and some haemorhagies).
Conclusions Monitoring clinical findings, organ involvement is important in patients with SSc, for therapy and prognosis. Observation of skin score, activity score, HAQ-DI and SHAQ score could help to control of activity and prognosis of patients with SSc. Capillaroscopy findings could help us to define stage of scleroderma and to find early stages of disease.
Disclosure of Interest None declared