[Immunopathological disturbances in systemic sclerosis].

Systemic sclerosis (SSc) is a disorder of the connective tissue characterized by the vascular dysfunction and progressive fibrosis of the skin, and various internal organs, which results in their dysfunction and failure. Activation of the immune system seems to play an important role in the pathogenesis of the disease. Contribution of the immune mechanisms to the development of the disease is indicated by the presence of the typical auto-antibodies, inflammatory cells infiltration in the skin and internal organs, and activation of cells participating in the immune response. Activation of the immune cells leads to release of proinflammatory/profibrotic cytokines and growth factors which in turn stimulate extensive proliferation of fibroblasts and collagen production. Activation of the immune system may also lead to impairment of endothelial function and development of angiopathy typical for systemic sclerosis, and subsequently chronic ischaemia of the affected tissues. In this review we describe the abnormalities of endothelial cells, T and B cells, and fibroblasts in patients with systemic sclerosis.