Rheopheresis as a causal therapy option for systemic scleroderma (SSc)

Abstract

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.

Autoantibodies as Biomarker and Therapeutic Target in Systemic Sclerosis

“…Therefore, rheopheresis was thought to have a beneficial effect on Raynaud’s phenomenon and digital ulcers. However, data regarding effects of rheopheresis on microcirculation in SSc are contradictory [ 193 , 194 ].…”

Rheopheresis: A narrative review

“…The targeted molecules are the fibrinogen as a plasma viscosity factor as well as a cluster of acute phase proteins such as the C-reactive protein and various cytokines, especially interleukin-6. 40…”

“…The targeted molecules are the fibrinogen as a plasma viscosity factor as well as cluster of acute phase proteins such as the C-reactive protein and various cytokines, especially interleukin-6. 40 In a female patient with diffuse systemic sclerosis resistant in a broad array of available treatment options Rh was performed at a rate of two sessions per week every 4 weeks for a total of 3 months. The treatment led to no effect on the total number of Raynaud attacks, significant reduction of the duration of Raynaud attacks from a median of 15 (5-45-95% CI 10-15) to 7 (3-30-95% CI 6-10; p < 0.01) minutes and a significant improvement of the Raynaud Condition Score from a median of 4 (3-4-95% CI 3-4) to 2 (1-3-95% CI 1-3-p < 0.01).…”

Rheopheresis for Digital Ulcers and Raynaud's Phenomenon in Systemic Sclerosis Refractory to Conventional Treatments

“…RheoP offers the potential advantage of positively influencing blood viscosity by removal of large plasma proteins, such as fibrinogen, which contribute critically to WBV. RheoP has been reported in SSc in a case report including two cases with severe DU (12). In this report, however, the effects on RP were not reported.…”