OBJECTIVES: To review the pathophysiological background of @entity785 in relation to the main components involved: microvascular system, immunological system and fibroblasts of the connective tissue. BACKGROUND: Although many particular aspects of the pathophysiology of @entity785 have been investigated in recent years, the complexity of the pathogenesis and the important links between the components involved remain unclear. METHODS: Literature review. RESULTS AND CONCLUSION: @entity2631 is a @entity2597 resulting in a progressive @entity82 of skin and internal organs. The genetic background is not clear. The microvascular system is one of the first targets involved (damage of capillaries, enhanced expression of adhesion molecules interacting with lymphocytes, perivascular infiltrates as starting points for tissue @entity82 ). The immune system is unbalanced (selection of T-cell subpopulations, elevated serum levels of several cytokines, occurrence of @entity18520 to topoisomerase I, centromeric proteins and RNA polymerases). As far as autoimmunity is concerned the triggering autoantigen is still unknown. Development of connective tissue @entity82 is prominent (subpopulations of fibroblasts with disturbed regulation of collagen turnover by TGF-beta, @entity12020 and collagen receptors (alpha1beta1, alpha2beta1)). Investigation of pathophysiology of @entity2631 is effected by monitoring the serum levels for soluble mediators, by cell culture studies of affected and non-affected fibroblasts and EC, by studying environmentally induced forms of @entity2631 and by studies using animal models.

Pathophysiology of XXXX : an update.

@entity2597 :: ('MESH:D003240', 'Disease') :: ['connective tissue disorder'] @entity82 :: ('MESH:D005355', 'Disease') :: ['fibrosis'] @entity18520 :: ('MESH:D001324', 'Chemical') :: ['autoantigens'] @entity2631 :: ('MESH:D012594', 'Disease') :: ['Scleroderma', 'scleroderma'] @entity12020 :: ('1490', 'Gene') :: ['CTGF'] @entity785 :: ('MESH:D012595', 'Disease') :: ['systemic sclerosis']
@entity2631 :: (MESH:D012594,Disease) :: ['scleroderma']
