[Etiopathogenesis of progressive systemic sclerosis].

Progressive systemic sclerosis (PSS) is a systemic disease of connective tissue involving the skin and internal organs characterized by fibrotic and degenerative changes. The disease is usually progressive, incurable and always leads to death. Several hypotheses about the etiopathogenesis of PSS exist which try to explain the cause of morphological changes of the connective tissue. The first theory advocates evolution of the primary lesion within the blood vessels, the second considers the primary event to be an abnormality of collagen metabolism manifested by fibrosis. The third postulates on an autoimmune basis for the disease, and the fourth is based on the disfunction of autonomic nervous system. All the theories are partly linked and represent the morphological, biochemical and immunological aspects of the pathogenesis of this disease.