Systemic sclerosis is considered by most researchers to be a disease characterized by early-stage damage to the endothelial layer of the microvascular system that eventually leads to fibrosis and the entire downstream symptom cascade. We introduce here a disease staging model for SSc that describes current treatment approaches for each stage of the disease process.
Therapeutic plasma exchange (TPE) is a procedure in which a large volume of plasma is replaced by a substitute fluid in a continuous flow process. A recent review of 46 published papers on the use of TPE as a treatment for SSc suggests that long-term TPE may offer a low-risk treatment alternative to current treatment approaches that focus on the use of immunosuppression.
Over the past 42 years, many published papers have documented that blood rheology is abnormal in patients with SSc. Individual papers have commented on or measured differing aspects of this abnormal rheology, including elevated whole blood viscosity (WBV), increased plasma viscosity (PV), decreased red blood cell (RBC) deformability, and abnormal RBC aggregation.