What is it about?
Currently, with the exception of autologous hematopoietic stem cell transplants (HSCT), no treatment has ever been shown to reverse the overall disease process in a patient diagnosed with systemic sclerosis (SSc). This case report is the first published to document the effects of therapeutic plasma exchange (TPE) as a sole intervention over a 22 year period. The patient went into full clinical remission over a two year period and has remained in full remission for the past 22 years as long as regular TPE is continued.
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Why is it important?
No conventional treatment approach has been shown to fundamentally alter the overall course of systemic sclerosis. At best they delay progression. This case report suggests that long-term TPE might be able to initially stop disease progression and potentially lead to gradual recovery of damaged organ systems over time.
Perspectives
This paper suggests that the most likely explanation for why TPE works is not temporary reduction of levels of plasma circulating pathogenic molecules such as autoantibodies, but rather that it normalizes blood rheology by eliminating red blood cell clumping and restoring normal overall blood viscosity. If RBC clumping directly or indirectly leads to the initial endothelial damage that is the hallmark of early SSc, eliminating this clumping and retaining normal blood rheology should prevent future damage to the microvascular system and the downstream development of fibrosis and systemic organ damage.
Edward S Harris
University of Wisconsin Madison
Read the Original
This page is a summary of: Successful long-term (22 year) treatment of limited scleroderma using therapeutic plasma exchange: Is blood rheology the key?, Clinical Hemorheology and Microcirculation, February 2017, IOS Press,
DOI: 10.3233/ch-16140.
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