{"id":165,"date":"2014-03-05T11:47:10","date_gmt":"2014-03-05T16:47:10","guid":{"rendered":"http:\/\/www.dysautonomiainternational.org\/blog\/wordpress\/?p=165"},"modified":"2014-03-06T07:40:07","modified_gmt":"2014-03-06T12:40:07","slug":"what-dysautonomia-patients-should-know-about-antiphospholipid-syndrome","status":"publish","type":"post","link":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/what-dysautonomia-patients-should-know-about-antiphospholipid-syndrome\/","title":{"rendered":"What Dysautonomia Patients Should Know About Antiphospholipid Syndrome"},"content":{"rendered":"

Many patients have sent Dysautonomia International questions about the association between antiphospholipid syndrome and\u00a0POTS, after an article appeared in the\u00a0medical journal\u00a0Lupus<\/em>\u00a0on this topic\u00a0on February 25, 2014. \u00a0Dysautonomia International asked the\u00a0first author\u00a0of this article, Dr. Jill Schofield, to address some of the questions raised by the patient community in the following blog post. \u00a0Please note that this is not meant to replace advice given by your own physician.<\/p>\n

What Dysautonomia Patients Should Know About Antiphospholipid Syndrome<\/strong>
\nby\u00a0Jill R. Schofield, MD<\/strong><\/p>\n

We have recently published the first clinical association of postural orthostatic tachycardia syndrome\u00a0(POTS),\u00a0neurocardiogenic syncope (NCS)\u00a0and\u00a0orthostatic hypotension (OH) with antiphospholipid syndrome (APS). \u00a0APS is also known as Hughes syndrome.\u00a0\u00a0I was\u00a0delighted to\u00a0co-author this paper with Professor Graham Hughes, who first described antiphospholipid syndrome in 1983,\u00a0Professor\u00a0Yehuda Shoenfeld, considered by many to be the \u201cfather of autoimmunity\u201d and Dr. Svetlana Blitshteyn, noted autonomic expert and member of the Dysautonomia International Medical Advisory Board. \u00a0You can view the abstract of the article here:\u00a0Postural tachycardia syndrome (POTS) and other autonomic disorders in antiphospholipid (Hughes) syndrome\u00a0(APS)<\/a>.\u00a0\u00a0The journal requires a paid subscription to view the full article, but your physician should be able to access it.<\/p>\n

What is APS?
\n<\/strong><\/span>APS is a complex autoimmune disorder that is associated with several different antiphospholipid antibodies. \u00a0These antibodies may be directed against\u00a0clotting factors, platelets,\u00a0and\/or the cells that line blood\u00a0vessel walls\u00a0and they cause the blood to be too sticky. This results in an increased risk of blood clots in:<\/p>\n

1) Arteries–causing most commonly stroke\u00a0or heart attack.
\n2) Veins–causing deep vein thrombosis (DVT) of the legs and\/or pulmonary embolus (PE) of the lungs.
\n3) Placenta–causing\u00a0recurrent\u00a0miscarriage, stillbirth or low birth weight babies.<\/p>\n

In addition to\u00a0an\u00a0increased risk for blood clots, a number of other manifestations may occur in APS due to “sludging”\u00a0of the blood.\u00a0The list of these non-clotting manifestations is long and they are less well known to most\u00a0physicians. \u00a0Some of these manifestations include migraine (which may be severe and refractory to usual treatments), memory loss, seizures and stress fractures. We have now demonstrated that POTS, NCS and OH\u00a0may also occur as non-clotting manifestations of APS.<\/p>\n

How is APS diagnosed?
\n<\/strong><\/span>The Sapporo criteria for the diagnosis of definite<\/strong> APS requires:<\/p>\n

1. Clinical criteria: Thrombosis (blood clot) or very specific pregnancy complications (such as three or more miscarriages).<\/p>\n

2. Laboratory criteria: Medium to high titer antiphospholipid antibodies on more than one occasion at least 12 weeks apart.<\/p>\n

These criteria were designed for rigorous clinical research studies, not for diagnosis. Unfortunately, most practicing physicians believe they were designed for diagnosis and this has resulted in patients with low titer antibodies and\/or non-clotting manifestations not being diagnosed with APS, when they do have the syndrome. My hope is that we can change this perception, because I believe that with earlier diagnosis, we can prevent<\/b> the thrombotic events!<\/p>\n

For our study, we used the following criteria:<\/p>\n

At least one clinical manifestation of the syndrome (including the non-clotting manifestations) along with\u00a0the presence of one or more of the antiphospholipid antibodies in any titer:<\/p>\n

1) Anticardiolipin IgG and\/or IgM
\n2) Beta 2 glycoprotein I IgG and\/or IgM
\n3) Lupus anticoagulant<\/p>\n

It is common to\u00a0have\u00a0only\u00a0one of these antibodies, but some patients have\u00a0two or even\u00a0all three. Occasionally various infections might cause a\u00a0transient\u00a0elevation in one or more of these tests, so the diagnostic criteria require you to have one or more of these antibodies on more than one occasion at least 12 weeks apart. \u00a0Notably, many of the patients in our study had low titers of the APS antibodies.<\/p>\n

The lupus anticoagulant test has a misleading name because it is not a test for lupus and it is associated with\u00a0increased\u00a0clotting, not decreased clotting as the name implies. APS, however, may occur along with lupus, as well as Sjogrens syndrome or rheumatoid arthritis.\u00a0It may also occur on its own.\u00a0Most APS experts are either\u00a0rheumatologists, hematologists\u00a0or obstetricians, but most physicians are familiar with these tests,\u00a0they can be ordered at any lab and\u00a0they are relatively inexpensive.<\/p>\n

Once a diagnosis of APS is made, there is no indication to repeat the antibody tests. \u00a0APS is not known to just resolve, but the antibodies are known to wax and wane over time. \u00a0There are many stories of patients who have had their levels fall into the normal range when their physicians repeatedly tested their antibodies and when told they no longer had APS and could stop their blood thinners, they went on to develop stroke or other major clotting events.<\/p>\n

Regarding imaging tests,\u00a0CT or MRI scans are used to test for stroke or other blood clots in\u00a0APS\u00a0patients with suspicious symptoms and clots in this syndrome can be found in any blood vessel. \u00a0 \u00a0Some\u00a0APS patients have “white spots” on brain\u00a0MRI scans; these are\u00a0felt to\u00a0represent\u00a0very tiny clots.\u00a0Ultrasound is\u00a0commonly\u00a0used to test for blood clots in the legs when there is\u00a0new leg pain and\/or swelling.<\/p>\n

How common is APS?
\n<\/strong><\/span>APS is not rare. \u00a0It has been estimated to affect approximately 1 out of 100\u00a0people\u00a0(1% of the population). \u00a0It is, however, underdiagnosed.<\/p>\n

We do not know how many POTS, NCS or OH patients have APS, but\u00a0Dysautonomia International recently funded a research project designed by Dr. Svetlana Blitshteyn to try\u00a0to shed\u00a0some light on the topic of autoimmune markers and autoimmune conditions in patients with POTS.\u00a0 Dysautonomia International will make an announcement when Dr. Blitshetyn’s study results are released.<\/p>\n

We also do not yet know how often autonomic disorders occur in APS patients.<\/p>\n

Should all patients with POTS be tested for APS?
\n<\/strong><\/span>Because this is a newly described clinical association, we have a lot to learn. \u00a0At this time, I\u00a0believe all POTS patients\u00a0should be tested for APS; other physicians might disagree. \u00a0At the very least, I believe all POTS patients with any of the following should be tested for APS: migraine, memory loss, balance trouble, livedo reticularis, Raynaud’s phenomenon, history of miscarriage,\u00a0another autoimmune condition,\u00a0a family history of blood clots\u00a0or a family history of autoimmune disease.\u00a0These were the most common findings in the patients in our study. \u00a0Also, of note,\u00a0three of the 15 APS patients included our study also had Joint Hypermobility Syndrome (JHS).<\/p>\n

\"Raynaud-hand2\"<\/a>\u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0\"Screen
\n<\/a>Raynaud’s Phenomenon \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 Livedo Reticularis<\/p>\n

The reason I believe\u00a0all POTS patients\u00a0without an apparent cause should be tested\u00a0for APS is\u00a0that POTS caused by APS\u00a0might improve with a trial of aspirin, clopidogrel, heparin,\u00a0warfarin\u00a0and\/or IVIG.\u00a0\u00a0Many of these agents increase\u00a0the risk of bleeding, however, which makes many physicians not experienced with APS nervous about using\u00a0them\u00a0in APS patients who have not had a clotting episode.\u00a0 Because APS is a very hypercoagulable condition, however, APS patients (even those on high doses of blood thinners) have a much greater risk of clotting than bleeding. Despite this, Professor Hughes believes that many APS patients have been under-treated due to physician concerns about bleeding.<\/p>\n

Additionally, APS is a serious medical condition and early diagnosis can help reduce the risk of major complications. \u00a0If you have one or more of the APS antibodies, you are at an increased risk for blood clots. If you\u00a0are aware you\u00a0have one\u00a0or more\u00a0of these antibodies, you can reduce your risk of blood clots by avoiding cigarettes, birth control pills\u00a0or hormone replacement\u00a0therapy. \u00a0You can also be sure that if you have other vascular risk factors, such as high blood pressure, high cholesterol or diabetes, they are treated aggressively. \u00a0Aspirin has also been shown to reduce the risk of arterial events in patients with APS antibodies. \u00a0Additionally, Professor Yehuda Shoenfeld’s research has shown that vitamin D levels are low (less than 15 ng\/ml) in half of patients with APS and that low levels are associated with an increased risk for clotting and non-clotting manifestations of the syndrome. \u00a0So it makes sense for these patients to also be treated with vitamin D.<\/p>\n

How is APS treated?<\/strong><\/span>
\nIn addition to the points made above, APS patients who have had an arterial or venous clotting event are generally treated with blood thinners (usually warfarin or heparin) for life. \u00a0There are also several newer oral anticoagulants, one of which is presently being studied in APS patients in London. \u00a0Until this data is available, these drugs are generally not recommended for most APS patients. \u00a0APS patients with recurrent miscarriages are treated with aspirin and usually heparin (warfarin is contraindicated in pregnancy) throughout their pregnancy and for at least 6 weeks after delivery.<\/p>\n

Importantly, Professor Hughes has found over the years that many of the non-clotting manifestations of APS often improve significantly or may even be completely aborted with anti-platelet agents such as aspirin or clopidogrel, and\/or warfarin or heparin. \u00a0He has also found this to be true for autonomic symptoms in some APS patients. \u00a0Two patients in our study with POTS that did not improve with standard APS treatments (despite improvement of other APS manifestations) improved significantly with regular intravenous immunoglobulin (IVIG) therapy. \u00a0Unfortunately,\u00a0IVIG is very expensive and many insurance companies require more data than two case reports before approving its use for a specific indication.<\/p>\n

Where can you find additional resources on APS?
\n<\/strong><\/span>Dysautonomia International has a brief explanation of APS on its\u00a0website<\/a>, as well as some links to APS related journal articles and non-profit organizations. You can find a physician experienced in APS by going to\u00a0APSAction.or<\/a>g, an international organization started in 2010 to improve collaboration amongst APS experts and to facilitate APS research. \u00a0An excellent patient forum on APS is\u00a0HealthUnlocked Hughes syndrome<\/a>\u00a0and Professor Hughes has written a great book for patients entitled,\u00a0Understanding Hughes Syndrome: Case Studies for Patients<\/a>.<\/p>\n

\"JillSchofield\"<\/a>Dr. Schofield is a Johns Hopkins trained internist who has developed an interest in APS over the last few years. She currently practices in Denver, Colorado but plans to develop a multi-disciplinary (i.e. involving physicians from many specialties) APS clinic in an academic environment and is currently exploring options for where best to do this.<\/span><\/em><\/p>\n\"Facebook\"<\/a>\"twitter\"<\/a>\"linkedin\"<\/a>\"youtube\"<\/a>\"vimeo\"<\/a>\"instagram\"<\/a>\"mail\"<\/a>by <\/span>\"feather\"<\/a>\"Facebook\"<\/a>\"twitter\"<\/a>\"reddit\"<\/a>\"linkedin\"<\/a>\"tumblr\"<\/a>\"mail\"<\/a>by <\/span>\"feather\"<\/a>","protected":false},"excerpt":{"rendered":"

Many patients have sent Dysautonomia International questions about the association between antiphospholipid syndrome and\u00a0POTS, after an article appeared in the\u00a0medical journal\u00a0Lupus\u00a0on this topic\u00a0on February 25, 2014. \u00a0Dysautonomia International asked the\u00a0first author\u00a0of this article, Dr. Jill Schofield, to address some of the questions raised by the patient community in the following blog post. \u00a0Please note that … Continue reading What Dysautonomia Patients Should Know About Antiphospholipid Syndrome<\/span> →<\/span><\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[12,3,13,14,20,4],"tags":[],"class_list":["post-165","post","type-post","status-publish","format-standard","hentry","category-autoimmune","category-dysautonomia","category-ncs","category-orthostatic-hypotension","category-pots","category-research"],"_links":{"self":[{"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/posts\/165","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/comments?post=165"}],"version-history":[{"count":12,"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/posts\/165\/revisions"}],"predecessor-version":[{"id":181,"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/posts\/165\/revisions\/181"}],"wp:attachment":[{"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/media?parent=165"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/categories?post=165"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/dysautonomiainternational.org\/blog\/wordpress\/wp-json\/wp\/v2\/tags?post=165"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}