[Immunology and clinical picture of progressive scleroderma]

The interrelations between clinic and immunology of systemic scleroderma serve the pathogenetic differentiation of the disease, on one hand, and the nosological classification, on the other. The primary cause of the pathogenesis of progressive scleroderma cannot be localized in the immune system. The fibroblast's activity, the ability of being stimulated and the metabolism are changed. The endothelial cell seems to be selectively influenced by serum proteases acting cytotoxically. The incidence of antinuclear antibodies reaches up to 90%, there is no correlation between titer and clinical activity. Circulating immune complexes are found with 30% of the patients, immunoglobulin and complementary deposits are found with 75%. The T-helper cell rate is increased, the T-suppressor cell population is decreased. Immunity phenomena permit the classification of three types, the definition of overlapping syndromes, as well as the estimation of visceral manifestations.