Affiliations: [a] Scleroderma Education Project Ltd, Madison, WI, USA
| [b]
Department of Physiology & Biophysics, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA
| [c]
Department of Clinical Pharmacology, University of Kansas Medical Center, Kansas City, KS, USA
| [d]
Department of Pathology and Laboratory Medicine, University of Wisconsin School of Medicine, Madison, WI, USA
Correspondence:
[*]
Corresponding author: Edward S. Harris, 2726 Van Hise Ave., Madison, WI 53705, USA. Tel.: +1 608 345 1984; E-mail: eharris@sclerodermainfo.org.
Abstract: While a number of studies have shown short-term beneficial effects of therapeutic plasma exchange (TPE) for treating systemic scleroderma (SSc), there have been no reports on the very long-term usage of TPE as the sole systemic treatment intervention. We report the case of a male patient, originally diagnosed with limited systemic scleroderma (lcSSc) in early 1990, who has been undergoing regular plasmapheresis treatments for more than 22 years, beginning in late 1993. Prior to commencing treatment, the patient exhibited symptoms including severe gastro esophageal reflux disease (GERD) with esophagitis, frequent Raynaud’s attacks, reduced lung function, and chronic chilling. With the exception of mild residual Raynaud’s, all of the patient’s symptoms reversed after three years of regular TPE treatments and he remains in complete remission. While the typical explanation for the therapeutic benefits seen with TPE focuses on temporary reduction of circulating antibodies or other pathogenic factors, we propose instead an explanation based on abnormal blood rheology as a novel disease pathogenesis model for SSc.
