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About Choclit

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  1. 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. 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. Please let me know if you have any questions. Ed
  2. The ACR research poster was actually just a spinoff from a paper that I presented at the American Society for Apheresis last May. It was a comprehensive review of the 40 published papers on the use of therapeutic plasma exchange to treat SSc. We are turning that into a manuscript now for submission to a journal soon. Here is the handout version of the review poster: http://www.sclerodermainfo.org/pdf/ASFA-Handout-US.pdf.
  3. Treatment of digital ulcers is very difficult and often unsuccessful. You may find this research poster that I presented last November at the American College of Rheumatology Meeting in DC interesting as it is directly related to this topic: http://sclerodermainfo.org/pdf/ACR-Handout-US.pdf.
  4. The term I use when describing limited systemic scleroderma is "slower progressing". The term "mild" is really not an accurate way of describing this variant of systemic scleroderma. I don't think anyone would describe MS as "mild". Limited systemic scleroderma is at least at this level, often worse.
  5. Joelf, that is a good point to emphasize. There are many people who have positive ANA and antibodies that never develop functional disease. Also, there are the 5% to 10% of people who are ANA negative by IFA who clearly meet diagnostic criteria for systemic sclerosis. I do, however, always suggest trying to figure out what specific antibody you have if possible as it gives you important information on risk profile and issues like whether or not certain medications are safe to use.
  6. Amanda, there are three different antibodies associated with diffuse systemic scleroderma: Scl-70, RNA Polymerase III, and U3-RNP. U3-RNP is very rare but the incidence of RNA Poly III and Scl-70 is about the same, around 20% of of the total SSc population. RNA Polymerase III has a higher risk of early kidney involvement than Scl-70 and as a result prednisone can be very dangerous with this antibody as it can trigger scleroderma renal crisis (the risk is dose dependent). It is also risky with Scl-70 antibodies. I am not sure if the research has been done with U3-RNP since this is a very rare diffuse antibody. Knowing your antibody is very important because of specific risks like this. Also, if you ANY variant of systemic scleroderma, you have internal organ involvement. I wrote this paper last year and it was reviewed and edited by one of the top scleroderma researchers in the US: http://sclerodermainfo.org/yes-you-do-have-internal-organ-involvement-but/. As the paper notes, the internal organ involvement may not lead to any functional impairment, but it is definitely there.
  7. The key here with your positive ANA results and symptoms is to get detailed antibody testing. There are 8 antibodies for scleroderma that can readily be tested for. Here is a list from the Antibodies section of the "Scleroderma FAQ" on my Scleroderma Education Project website (sclerodermainfo.org): http://sclerodermainfo.org/faq/scleroderma-antibodies/. The three most common antibodies in systemic scleroderma are Scl-70, centromere, and RNA Polymerase III. With a standard ENA panel you were probably only tested for Scl-70 antibodies but some do include centromere, depending on the testing method. If those are negative, then go on the other five antibodies listed in the antibody table referenced above.
  8. About longevity with limited systemic scleroderma... Research shows that patients with this disease variant, usually associated with anticentromere antibodies but also rarely with anti-Th/To antibodies, tend to live relatively normal life spans but with steadily increasing disability over time. The most dangerous complication with centromere antibodies, in particular, is pulmonary arterial hypertension (PAH) which eventually occurs in about 20% to 25% of patients with limited scleroderma. Most other patients will develop some impairment in lung functioning over time but in most cases will not progress to PAH. In rare cases, patients develop scleroderma renal crisis, which used to be a major complication, but now if caught early, this is usually very treatable with ACE inhibitors so it is no longer a leading cause of death in patients with scleroderma.
  9. Can you indicate which antibodies you were tested for and which one was positive? There are 10 separate antibodies currently identified with systemic scleroderma, as indicated in the Antibody section of the Scleroderma FAQ: http://sclerodermainfo.org/faq/scleroderma-antibodies/.
  10. Are you diagnosed with systemic scleroderma? Do you know your blood type? Last summer, the Scleroderma Education Project, a 501c3 non-profit organization focused on scleroderma education and research, conducted a preliminary survey of blood types among patients with diagnosed systemic scleroderma. We ended with 743 responses and some very interesting results. We are now working with statisticians at the University of Wisconsin in Madison to do the data analysis needed for the paper that we will be writing to discuss our findings. In the original survey, we did not ask about country of birth. It turns out that blood type distributions are different in different countries and in order to do the best possible statistical analysis, we need to adjust the data based on the distribution of responses by country. So, we are redoing the survey with just two questions: 1) What is your blood type? 2) What is your country of birth? If you are formally diagnosed with systemic scleroderma (not morphea or linear scleroderma) and you know your blood type, please take this anonymous two question survey: https://www.surveymonkey.com/r/SN2ZSQ6. It will take less than a minute. Please take this new survey even if you filled out the previous survey last summer. Also, please ask any of your scleroderma friends to take the survey as well. And, if you are a member of a scleroderma group or organization, see if you can get them to publicize this survey as well. In addition to clicking the link above, you can go directly to the Scleroderma Education Project home page at www.SclerodermaInfo.org and click the link at the top of the page. The survey will end on March 31st. Thank you for helping with this quick research study.
  11. The Scleroderma Education Project (SclerodermaInfo.org) is a 501c3 non-profit organization focused on: 1) providing up-to-date, research-based educational information on systemic scleroderma diagnosis and treatments, and 2) advancing new research focused on understanding the role of abnormal blood rheology in systemic scleroderma pathogenesis. As part of our research efforts, we are conducting a brief survey on blood type distribution in patients with diagnosed systemic scleroderma. If you are formally diagnosed with systemic scleroderma, including diffuse scleroderma, limited scleroderma, CREST, or overlap syndromes such as Mixed Connective Tissue Disorder (MCTD) AND you know your blood type, we would greatly appreciate if you would take one minute to fill out an anonymous, two question survey. Here is a link to the survey: https://www.surveymonkey.com/r/SN2ZSQ6. The survey will conclude on March 31st. We are working with statisticians at the University of Wisconsin in Madison, WI and expect to publish our findings some time this summer. Thank you for your participation in this research study.
  12. This is the title of a new educational article on my Scleroderma Education Project website (SclerodermaInfo.org). Yes, You DO Have Internal Organ Involvement, But… Note: if you sign up on the Scleroderma Education Project home page, you will automatically be notified whenever any new posts are added to the website.
  13. I just reviewed the most recent literature and it gives a range for the US and Europe of from 150 to 300 per million population which gives a range of 48,000 to 96,000 cases. The studies are too inconsistent in methodology to see any trends.
  14. The research data from Mayes (2003) suggests that in the US there are about 58,000 cases of systemic scleroderma and 80% are female. I always blame my limited scleroderma diagnosis on the fact that I am a feminist! :-)
  15. It is my understanding that while this is not completely consistent among scleroderma researchers, the new trend is to use antibodies as the primary determinant of scleroderma subtype. Historically, only two antibodies where know to be associated with scleroderma: centromere and Scl-70. Patients with centromere antibodies typically have later skin involvement and it is almost always "limited" to hands (sometimes up to the elbows), feet, and face. In contrast, patients with Scl-70 antibodies have skin changes that were more "diffuse", including the trunk. This is where the names Limited Cutaneous Systemic Scleroderma (lcSSc) and Diffuse Cutaneous Systemic Scleroderma (dcSSc) comes from. When the RNA Polymerase III antibody was identified, it also has diffuse skin changes. Later Th/To antibodies were identified as another rare scleroderma related antibody and the skin pattern changes were similar to centromere. Now there are about eight different scleroderma related antibodies plus about 6% of patients with clear scleroderma symptoms but test ANA negative even by the IFA method. They are divided into three "camps": diffuse, limited, and overlap syndromes (such as Mixed Connective Tissue Disorder). Antibody type doesn't change and it is very rare (less than 2%) for a patient to actually have more than one scleroderma related antibody. Some clinicians still look at the skin changes as diagnostic, which leads to a great deal of confusion where patients are told that they are limited but later changed to diffuse as skin changes progress. I am in complete agreement with the modern trend of using antibodies as the primary classification method. Here is a link to an antibody table within my Scleroderma FAQ that lists Scleroderma related antibodies and how they are classified, as well as some significant information about risks with each type.
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