Successful long-term (22 year) treatment of limited scleroderma using therapeutic plasma exchange: Is blood rheology the key?

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.

Therapeutic plasma exchange for the treatment of systemic sclerosis: A comprehensive review and analysis

“…Three of the CRs are notable in that they reported on the results of long-term, regular TPE as a systemic treatment approach. 40 , 43 , 49 Eight of the CRs used TPE as the sole treatment intervention. Of the CRs, 12 received a Grade I rating; however, only 4 of these used TPE as the sole treatment intervention.…”

“…A 2017 very long-term (22-year) CR 40 documented the effects of regular TPE as the sole systemic intervention in a patient with rapidly progressing anti-centromere-positive lcSSc. TPE was administered in a pulsed protocol (one TPE treatment per week for 4 weeks and 8 weeks with no TPE, and the procedure was repeated).…”

“…To date, no medications have proven to be very effective in treating either pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD), and as a result, lung-related complications from both PAH and pulmonary fibrosis (PF) are the leading causes of SSc-related mortality. 1 According to a recent study, 2 it is not clear that any standard treatment for SSc has led to improved SSc survival rates over the past 40 years, beyond what would be expected by overall improvements in survival rates in the general population during this same time period.…”

Rheopheresis for Digital Ulcers and Raynaud's Phenomenon in Systemic Sclerosis Refractory to Conventional Treatments

“…Other protocols for TPE based on single cases with 4 weekly treatments (15) or small studies with ten to 12 weekly treatments (9–11) have been proposed. For logistic and practical reasons, we decided to use the protocol described above, which was acceptable for the patient and could be implemented easily into practice.…”

The role of plasma exchange in the management of autoimmune disorders

“…In 10 patients with SRC treated with TPE — due to concomitant MAHA or intolerance to high doses of ACEi — we observed a significant decrease in 5‐year mortality versus 10 patients treated with ACE‐i alone (Cozzi et al , ; Zanatta et al , ). A recent literature review by Harris et al () on the role of TPE in SSc suggested that long‐term TPE may offer a low‐risk way to control and, in some cases, reverse SSc symptoms. However, the role of TPE in the management of SSc is still not well established (2016 ASFA‐G category III).…”