Review: Therapeutic Plasma Exchange (Plasmapheresis) to Treat Systemic Sclerosis (Scleroderma)

What is it about?

Systemic sclerosis (SSc/scleroderma) affects approximately 60,000 to 100,000 people in the U.S. and has the single highest mortality rate of any autoimmune rheumatic disease. In spite of the grave threat posed by this diagnosis, there is no FDA-approved therapy for systemic sclerosis. Therapeutic plasma exchange (TPE), often called plasmapheresis, is a widely used treatment for a number of diseases. This paper is a comprehensive review of 46 published papers on the use of TPE to treat systemic sclerosis. In almost all studies, the majority of patients receiving TPE showed improvements in both symptoms and laboratory markers, whether in short-term treatment for crisis situations or from long-term administration of regular TPE. The review concludes that "… in contrast to current immunosuppressive treatments that carry significant risk, long-term TPE appears to be safe, well-tolerated, and associated with only very few, mostly minor side effects. While TPE is not an inexpensive procedure, annual costs are similar to modern pharmaceuticals commonly used to treat SSc and other autoimmune diseases."

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Why is it important?

Current treatments for systemic sclerosis are focused on individual symptoms or overall suppression of the immune system. Unfortunately, the research clearly demonstrates that none of these treatments are very effective. In fact, a recent study documents that there has been no improvement in overall mortality rates in systemic sclerosis over the past 40 years beyond what is also seen in the general population. While the research on TPE is far from conclusive since modern, well-designed randomized controlled trials have not been done, TPE offers an alternative that clinicians may want to consider, especially given its overall excellent safety profile and costs that are similar to standard pharmaceutical treatments often used in treating other autoimmune diseases.

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