The articles in this section of the Scleroderma Education Project website supplement the information included in the patient-focused Scleroderma FAQ™ and Guide for New and Future Patients™. In some cases, the articles are intended for a different audience (e.g., the Guide for Family and Friends). In other cases, the articles explore topics introduced in the FAQ or Guide in significantly more detail and in some cases with a more technical focus. These more technical articles include appropriate research citations, allowing interested readers to examine the research literature behind these articles. Additional articles will be added in the future. Listed below are summaries of all of the articles available in this section of the website. These articles are formatted as printable PDF documents in both US and A4 formats.
Supplemental Patient Education Articles
Guide for Families and Friends™
(Coming Soon) This information in this article is mostly extracted from the longer Guide for New and Future Patients™, but has been refocused to help the Scleroderma patient’s family and friends better understand the disease and what s/he is going through. It includes an overview of Scleroderma including typical symptoms and disease course. It also discusses the major difficulties and frustrations that many Scleroderma patients go through before finally being diagnosed, often years after first seeing their doctor with initial symptoms of the disease. It also helps family members and friends better understand what the patient is going through so they can be more supportive and help the patient better deal with this life-changing disease.
ANA and Antibody Series
Scleroderma ANA and Antibody Testing Basics
This technical article discusses issues related to ANA and antibody testing for patients that have or might have Scleroderma. It includes a discussion of research that shows that current testing methods commonly used for ANA and antibody screening have a high likelihood of falsely indicating a negative ANA result and missing key Scleroderma-related antibodies, often delaying diagnosis and treatment unnecessarily for years.
How to Do Scleroderma ANA and Antibody Testing Correctly
This article, written for clinicians, is a bullet point summary version of the information found in the Scleroderma ANA and Antibody Testing article. It briefly covers the problems resulting from changes in ANA testing methodology and details how to most effectively do appropriate ANA and antibody testing to help in clinical diagnosis of patients that may have systemic scleroderma or a scleroderma overlap syndrome.
Changes in ANA and Antibody Levels In Scleroderma
This article was prompted by a question asked by a patient through this website. It discusses the research on when and how ANA and antibody levels change over time and the clinical significance of these changes.
The Puzzle of ANA-Negative Systemic Scleroderma
Links: US Version A4 Version Research studies consistently show that between 5% and 10% of patients with clear systemic scleroderma symptoms repeatedly test negative for ANA when done by the “gold standard” IFA method. This paper discusses this unusual situation.
ANA and Antibody Test: Why the Testing Lab Matters (coming soon)
Hyperviscosity and Therapeutic Plasma Exchange Series
(Note: Some of the articles in this section of the Scleroderma Education Project reflect the views of this author and may not necessarily be representative of the views of medical community at large.)
Successful Long-Term (22 Year) Treatment of Limited Scleroderma Using Therapeutic Plasma Exchange: Is Blood Rheology the Key?
Note: This case report is in press at Clinical Hemorheology and Microcirculation
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
Note: This is an extended version of a poser presented at the American Society for Apheresis conference in May 2016
Abstract: This research poster was a review and analysis of 40 published research papers on the user of therapeutic plasma exchange (TPE) to treat systemic scleroderma. The reviewed articles included a total of 533 patients. Fifteen of these were case studies; the rest ranged from small observational studies to prospective clinical trials with control groups. Here is a summary of the key findings from this comprehensive review:
- TPE was very well tolerated by almost all patients. Adverse events were very rare and in almost all cases mild.
- In all studies, the majority of patients receiving TPE showed improvements in both symptoms and laboratory markers, whether in short-term treatment of crisis situations or from long-term administration of regular TPE.
- Many patients experienced significant improvement in Raynaud’s symptoms and initial healing of digital ulceration after just 3 to 4 weekly treatments.
- While the effects of even a few TPE treatments often lasted for a number of months, only continued long-term treatments resulted in stabilization of symptoms or in one recent case report, sustained remission over a 21-year period.
- Venous access problems occurred in a significant minority of patients receiving long-term TPE, leading to cessation of TPE treatments in some cases and switching to central venous access in other cases.
In contrast to current treatment modalities such as immunosuppression that carry significant risk and show limited efficacy, the results shown in the clinical studies reviewed for this article suggest that long-term TPE may offer a low-risk way to control and in some cases reverse systemic scleroderma.
Scleroderma Blood Hyperviscosity: Implications for Research and Treatment
There is a significant body of research that documents blood hyperviscosity (thickness) in Scleroderma patients that appears to be caused by red blood cell hyperaggregation (clumping). This technical article discusses the potential implications for this as a possible trigger for the cascade of symptoms that develop in Scleroderma patients. It suggests several possible research studies that can test the validity and significance of this disease pathogenesis hypothesis. The article also reviews several treatment approaches that can potentially break up the red blood cell clumping and may lead to the development of drugs or other treatment approaches that target a different part of the Scleroderma disease process than is targeted by current treatments.
Suggested Protocol for a One-Year Trial of Therapeutic Plasma Exchange for Treating Patients with Limited Systemic Sclerosis
The purpose of this document is to provide background information for physicians 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 well-designed clinical trials of TPE for treating patients with SSc.