I am very happy to announce that our review paper titled “Therapeutic Plasma Exchange for the Treatment of Systemic Sclerosis: A Comprehensive Review and Analysis” was just accepted for publication by the “Journal of Scleroderma and Related Disorders”. JSRD is the top research journal in this field and is read by most researchers and clinicians who are focused on systemic sclerosis (scleroderma). My co-authors (all MDs or PhDs) have expertise in plasma exchange, rheumatology, blood rheology (physical properties of blood), and immunology. This paper is an expanded version of two research posters that I presented at the American Society for Apheresis and American College of Rheumatology annual meetings during 2016.
Here is the abstract of the paper:
Background. Therapeutic plasma exchange (TPE) has been tried as a treatment approach for systemic sclerosis (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.
I do not yet have the timeline for publication of this paper. It will be published online initially and later in print. Typically, the time period between acceptance of papers by JSRD and online publication is a couple of months. However, this is a very large paper (5500 words, 8 tables, 97 references) so it may take longer to get through the final editing process. The article will be published open access so that as soon as it is published online, anyone, anywhere will be able to download it free of charge.
Many of you here are familiar with my backstory (http://sclerodermainfo.org/pdf/Ed.pdf) and the fact that I have been receiving regular TPE treatments since 1993. My own case report was published last year (http://www.sclerodermainfo.org/pdf/CHM-Case-Report-Manuscript-US.pdf). As a result, a number of you have expressed interest in talking with your doctors about whether or not TPE might be an appropriate option for you to consider. Some of you have already tried to talk to your clinicians about this with little success, in part because most rheumatologists, even scleroderma specialists, are almost completely unfamiliar with the research literature on TPE (mostly done outside the US). In addition, rheumatologists are generally unfamiliar with TPE as it is little used for the diseases they deal with on a routine basis. (TPE is much more commonly used in other fields such as neurology.) Because of this, they often have incorrect ideas about issues such as safety, cost, and venous access with TPE.
If this is something you are considering, once the paper is published and available for download, it may be helpful in educating your clinicians about TPE. Based on comments and requests from a number of you, my current plan is that when the paper is published, I will have available a short, bullet-point overview of TPE as a potential treatment option that you can give to your clinicians as a starting point for these discussions. It will include links to the following more detailed articles:
- Successful Long-Term (22 Year) Treatment of Limited Scleroderma Using Therapeutic Plasma Exchange: Is Blood Rheology the Key?. This case report is the only long-term published paper on the use of TPE as the sole systemic treatment intervention over a very long time period.
- Therapeutic Plasma Exchange for the Treatment of Systemic Sclerosis: A Comprehensive Review and Analysis. In addition to the formal review of 46 published papers on the use of TPE as a treatment for systemic sclerosis, this paper has detailed discussions of common clinician concerns such as safety, cost, and venous access.
- Suggested Protocol for a One-Year Trial of Therapeutic Plasma Exchange for Treating Systemic Sclerosis. This document gives a starting point for clinicians to use if they decide to move forward with a TPE trial. It also includes suggested subjective and objective measures that can be helpful in monitoring treatment effectiveness.
- Therapeutic Plasma Exchange: A Guide for Newbies. This is actually a document for patients who are about to start TPE and gives suggestions on how to make the TPE treatment experience as successful and comfortable as possible.
It is important for anyone considering trying a treatment like TPE to realize that this is an experimental treatment. High quality, randomized controlled trials of TPE as a treatment for systemic sclerosis have not yet been done. While the overall safety profile of TPE is considered excellent, it is not absolutely risk-free, although these risks are substantially less severe that those associated with other treatments such as autologous bone marrow transplants. It is also important to realize that since systemic sclerosis is a chronic disease, TPE, similar to most other treatments, needs to be continued on a permanent basis until therapies targeting the currently unknown underlying biological mechanisms of this disease are developed.
Let me know if you have any questions.
Note: this post has been approved by the Medical Advisory Board of the Scleroderma Education Project.