Indexed on: 19 Oct '17Published on: 19 Oct '17Published in: Clinical hemorheology and microcirculation
A complex pathomechanism accounts systemic sclerosis as a form of collagenosis. A triad of vasculopathy, autoinflammation, and dysbalance of the fibroblast function can be seen as cause, as well as symptomatic appearance. Comparative with other collagenoses, e.g. Lupus erythematosus, vasculopathy, instead of autoinflammation, appears to be clinically important in systemic scleroderma. The fact that autoinflammation does not represent the major role in the maintenance of the disease is also evident by the lack of therapeutic effects of classical systemic immunosuppressants. Therapeutic approaches with regard to vasculopathy show better effects. In consideration of therapeutic options, such principles are therefore most important. Apheretic methods filter out plasma proteins in the sense of plasmapheresis. Fibrinogen as a plasma viscosity factor is predominantly targeted and filtered out. In addition other accompanying plasma proteins are also reduced. This occurs on the one hand by dilution effects and on the other by unspecific binding. By this mechanism, acute phase proteins such as the C-reactive protein and various cytokines, especially interleukin-6 are reduced by this method. Looking more closely at these random adjunctive plasma proteins, a possible central role of interleukin-6 in the development and maintenance cascade of systemic scleroderma becomes clear.