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Successful Long-Term (22 Year) Treatment of Limited Scleroderma Using Therapeutic Plasma Exchange: Is Blood Rheology the Key?

Harris ES, Meiselman HJ, Moriarty PM, Weiss J. Successful Long-Term (22 Year) Treatment of Limited Scleroderma Using Therapeutic Plasma Exchange: Is Blood Rheology the Key? Clin Hemorheol Microcirc. 2017;65:131-136. doi:10.3233/CH-16140.

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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 Scleroderma: A Comprehensive Review and Analysis

Harris ES, Meiselman HJ, Moriarty PM, Metzger A, Malkovsky M. Therapeutic plasma exchange for the treatment of systemic sclerosis: A comprehensive review and analysis. J Scleroderma Relat Disord. 0(0):2397198318758606. doi:10.1177/2397198318758606.

JSRD: TPE/SSc Review

Abstract

Background. 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 could lead to symptom improvement. Based on our impression that clinicians and researchers are largely unaware that a large volume of research has been published about the use of TPE as a treatment for SSc, we conducted a comprehensive review and analysis of all published research on this topic.

Results. We identified 46 relevant articles that met our search criteria, involving a total of 572 patients. Nineteen of these were case studies; the rest ranged from small observational studies to prospective randomized clinical trials.

In all but two studies, most patients receiving TPE showed improvements in both clinical symptoms and laboratory markers, including significant improvement in Raynaud’s symptoms and healing of digital ulceration after three to four weekly treatments.  The beneficial effects from even a short course of TPE treatments were long-lasting, typically six months or longer.  TPE was very well tolerated.  Adverse events were rare and, in almost all cases, mild and transitory.

Conclusion. These results suggest that long-term TPE may offer a low-risk way to control and in some cases reverse SSc symptoms.  The mechanism for the clinical improvements seen from TPE in SSc patients is unclear.  Therefore, additional studies of TPE effects in SSc appear to be highly desirable.

Suggested Protocol for a One-Year Trial of Therapeutic Plasma Exchange for Treating Systemic Sclerosis

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The purpose of this document is to provide background information for clinicians and patients who have reviewed the research literature on the use of therapeutic plasma exchange (TPE) to treat systemic sclerosis (SSc) and made the determination that this may be an appropriate treatment option. While this is not intended to be a formal research proposal, it is hoped that by following a standardized protocol for administering TPE, as well as including a basic set of objective measures during the trial of TPE, we may be able to glean important information that can be used as the basis for future clinical trials of TPE for treating patients with SSc.

Therapeutic Plasma Exchange: A Guide for Newbies

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Therapeutic plasma exchange (TPE) is a procedure used to treat a variety of diseases and conditions where something circulating in the blood is causing or contributing to the disease process. TPE can be short-term, only requiring a limited series of TPE treatments to deal with an acute condition (for example, Guillain-Barre syndrome), or it can be required for a long- term or even life-long basis for some chronic conditions (for example, multiple sclerosis, myasthenia gravis, or systemic scleroderma).

Starting a new, unknown treatment like TPE can be scary and may lead to anxiety that can actually make it more difficult to perform TPE successfully. The goal of this patient-focused document is to help to explain exactly what TPE is and how it is performed, how to prepare for a TPE treatment in order to make it as easy and successful as possible, what to expect during a TPE treatment, how to make yourself as comfortable as possible while undergoing TPE, and how you can expect to feel once a TPE treatment is completed.