Long-Term (22 Year) Successful Treatment of Limited Systemic Scleroderma Using Plasmapheresis

While a number of studies have shown short-term beneficial effects of plasmapheresis (PA) for treating systemic scleroderma (SSc), there have been no reports on the very long-term usage of PA as the sole systemic treatment intervention. We report on 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 PA treatments and he remains in complete remission. While the typical explanation for the therapeutic benefits seen with PA focuses on temporary reduction of circulating antibodies or other potential pathogenic factors, we propose instead an alternative explanation based on a novel disease pathogenesis model for SSc.