Therapeutic Plasma Exchange for the Treatment of Systemic Scleroderma A Comprehensive Review and Analysis
Purpose. Systemic scleroderma (SSc) is a family of rare autoimmune disease that primarily affects middle age women. It is disabling, disfiguring, and steadily progressive, attacking internal organs through fibrotic processes in addition to its characteristic skin changes. Current treatment approaches focus on using immunosuppressants to slow the disease process plus interventions targeted at specific symptoms. Neither approach is currently very effective. Therapeutic plasma exchange (TPE) has been tried as a treatment approach for SSc since 1978 based on the rationale that some circulating factor is involved in disease pathogenesis, e.g. autoantibodies or immune complexes, and that removing the potential pathogenic factors should lead to symptom improvement. Method. A comprehensive review of the research literature was conducted during November and December 2015. Additional articles were found by reviewing all of the references in the original article list. Articles written in other languages were included only if the abstracts were in English. Results. We identified 40 relevant articles that met our search criteria, involving a total of 533 patients. 15 of these were case studies; the rest ranged from small observational studies to prospective clinical trials with control groups. Because of the very diverse nature of the included studies and the greatly varying protocols, it is difficult to provide quantitative summary data. However, a number of very clear observations can be made upon careful review of these articles. TPE was very well tolerated by almost all patients. Adverse events were very rare and in almost all cases mild. In all studies, the majority of patients receiving TPE showed improvements in both symptoms and laboratory markers, whether in short term treatment of crisis situations or from long term administration of regular TPE. Many patients experienced significant improvement in Raynaud's symptoms and initial healing of digital ulceration after just 3 to 4 weekly treatments. While the effects of even a few TPE treatments often lasted for a number of months, only continued long term treatments resulted in stabilization of symptoms or in one recent case report, sustained remission over a 21 year period. Venous access problems occurred in a significant minority of patients receiving long term TPE, leading to cessation of TPE treatments in some cases and switching to central venous access in other cases. Conclusion. In contrast to current treatment modalities such as immunosuppression that carry significant risk and show limited efficacy, the results shown in the clinical studies reviewed for this article suggest that long term TPE may offer a low risk way to control and in some cases reverse SSc 1 Scleroderma Education Project, Madison, WI. (Corresponding author: eharris@sclerodermainfo.org) 2 Keck School of Medicine, University of Southern California, Los Angeles, CA 3 RDL Reference Laboratory, Los Angeles, CA 4 University of Kansas Medical Center, Kansas City, KS