Sep 5, 2011

What is Sjogren's Syndrome and how can it cause POTS?

Last month I was diagnosed with Sjogren's Syndrome by Cleveland Clinic.  I was relieved to finally know what was making me so sick.  My diagnosis breathed new hope into me, because now that we know what is going on, maybe we can treat it and reverse it.  I announced my diagnosis to friends and family with phone calls and Facebook posts, and lots of them asked me questions about Sjogren's and some said, "[s]o does that mean you don't have POTS?" Unfortunately, no, I still have POTS.   Sjogren's, as I will explain below, is causing my POTS - but if we can treat my Sjogren's, maybe we can improve my POTS symptoms.

A few weeks after I announced my diagnosis to the world via Facebook, superstar tennis pro Venus Williams, age 31, announced to the world via press release that she was recently diagnosed with Sjogren's Syndrome, and would have to withdraw from the U.S. Open tennis tournament.  I am sad for Venus, because she is such an amazing woman and wonderful to watch on the court.  However, I am glad she was brave enough to share her story with the world.  Sjogren's is one of those diseases that millions of people have, but the general public for the most part has never heard of it.  Venus is the first celebrity to tell the world she has Sjogren's (unless you count the woman who played Erin on the Walton's - dating myself here!) and this of course has all of the press talking about Sjogren's. 

Venus's diagnosis and having to pull out of the tournament is shocking to many people, because as a professional athlete, she is in peak physical condition and is by no means wimp or whiner when it comes to aches and pains.  I think this should help explain to those who are not familiar with Sjogren's, how much this disease can really debilitate someone and impact every aspect of their life. If Sjogren's is kicking Venus Williams in the arse and making her feel sick all the time, imagine what it is doing to someone who isn't in peak physical form and who doesn't have access to the best doctors and medicine money can buy.

You may read a Sjogren's related website or an article in the newspaper about Venus talking about Sjogren's patients having dry eyes and dry mouth.  While dry eyes and dry mouth are a distinguishing feature of Sjogren's that help doctors differentiate it from other diseases, the dry eyes and dry mouth are just the tip of the iceberg.  I think most Sjogren's patients would be happy to just have dry eyes and dry mouth - most patients have many other symptoms that are much more serious than dry eyes and dry mouth.  Also, the dry eyes that accompany Sjogren's can sometimes lead to severe ulceration of the eye, infection, and eventually blindness.  The dry mouth can lead to severe canker sores - sometimes 5 or more in the mouth at all times - which can make eating painful.  The dry mouth can also lead to digestive problems, because saliva is essential for proper digestion.  Dry mouth can also lead to choking on food, trouble swallowing, increased lung infections from inhaling ones food (coupled with dry lungs caused by Sjogren's), tons of cavities and dental infections (even if you brush and floss all the time), and increased sensitivity/allergic responses to many foods.  So even if dry eyes and dry mouth were the only symptoms, it's not like any dry eyes or dry mouth you might have after a day of drinking too much alcohol in the sun.

The primary mechanism of Sjogren's Syndrome is when your body's own white blood cells (lymphocytes) attack and invade your exocrine glands.  Exocrine glands are moisture producing glands found throughout your body.  Saliva glands and tear glands are exocrine glands, and thus when Sjogren's causes lymphocytes to damage those glands, you end up with dry eyes and dry mouth.  Exocrine glands are also found in the esophagus, stomach and pancreas, to aid with production of digestive juices. Sweat glands on the skin are also exocrine glands, which help cool down your body and rid the body of toxins.  There are exocrine glands in your sinuses, lungs and vocal cords, which moisturize the mucus membranes in those areas to protect you from infections and particulates like smoke and pollen.  There are exocrine glands in the female and male genitalia, to assist with lubrication during sexual intercourse, which help prevent infections and physical damage to the area.

So what happens when Sjogren's damages some or all of those exocrine glands? 

The lungs can become dry and easily irritated, exacerbating asthma, causing shortness of breath, causing increased infections, increased sensitivity to particulates like smoke and pollen and frequent dry coughing.  Sjogren's can lead to recurrent bronchitits, pneumonitis and interstitial lung disease.  Dry stuffy sinuses, frequent nosebleeds, sinus infections and recurrent sinusitis can occur.

Frequent and long lasting canker sores, oral infections, cavities, receding gums, the need for dentures at a young age, difficulty speaking and swallowing, choking on food, swelling of the salivary glands, so much so that the patient may have large lumps on the side of the cheeks.

Acid reflux, GERD, heartburn, esophagitis, stomach upset, gastroparesis (an abnormal slowing down of digestive movement thru the bowels), cramps, nausea, autoimmune pancreatitis

Dry skin, vasculitis (swelling of the veins which can be painful), Raynaud's Phenomenon (severe pain and skin color changes in hands/feet with exposure to cold)

Dry eyes, scratchy eyes, itchy eyes, eyelids sticking to eyeballs, crusty eyes, searing stinging pain in eyes, blurred vision, ulcerations of the eye and frequent eye infections.

Vaginal dryness, painful sexual intercourse, irritation, itching or swelling after sexual intercourse, more frequent yeast infections.

Swollen liver which can cause abdominal pain, abnormal liver function tests, chronic active autoimmune hepatitis, primary bilary cirrhosis.

Sjogren's can also manifest in ways that are not directly correlated to exocrine gland damage.  There are other inflammatory processes involved in Sjogren's and researchers are still trying to identify the exact physiological mechanisms by which these "extraglandular" symptoms occur.

Arthritis, joint pain, joint swelling and inflammation, muscle pain, fibromyalgia like symptoms.

I gave this a category unto itself because it doesn't fit neatly into any other category.  Fatigue is one of the most common symptoms of Sjogren's.  It is a fatigue that is not relieved by rest and sleeping.  You can sleep well for 10 hours, and wake up feeling like your just got run over by a truck.  Some patients report feeling good one day, and the awful the next, or good one week, and then awful the next - this cyclic presentation is common in many autoimmune diseases.  The best description I have heard is someone who said it feels like you have the flu everyday, or it feels like you have been beaten up by a thug.  When the fatigue hits me bad, I feel like I have cement shoes on and bowling balls tied to my arms and head.  It takes great effort just to get out of bed or stand up from a chair.

Central nervous system complications can result in spinal and brain lesions, cognitive/memory impairment (brain fog), painful peripheral neuropathy (causing by damage to sensory small fiber nerves in the feet and other extremity areas), motor neuropathy (damage to nerves that control muscles, which can impair ones ability to walk or use ones arms), and autonomic neuropathy (damage to the nerves that control heart rate, blood pressure blood volume, breathing, digestion and other essential involuntarily bodily functions - autonomic neuropathy can cause POTS).  Sjogren's can damage any nerve in the body, but scientists haven't figure out the precise mechanism by which this occurs.  Some theorize that vasculitis (swelling of the blood vessels) presses on the nerves and eventually causes them to be squeezed to hard they lose function.  There is other evidence that alpha-fodrin antibodies directly attack the nerves in some Sjogren's patients, and that this frequently appears before the onset of the more traditional dry eyes/dry mouth exocrine gland symptoms.

Sjogren's can be divided into Primary and Secondary forms. People with the Primary form just have Sjogren's, but are more likely present at a later point in their life with another autoimmune disease compared to the general public.  Secondary Sjogren's is when someone has Sjogren's secondary to another autoimmune disease, such as lupus or rheumatoid arthritis.  For many patients with autoimmune diseases, over time if they develop one, they are more likely to develop a few more as well.

Women with Sjogren's, are more likely than healthy women to miscarry during pregnancy.  This is because anti-phospholipid anitbodies, which are associated with Sjogren's and other autoimmune diseases, tend to cause abnormal blood clotting, which can clot in the placenta and injure the fetus.  If anti-phospholipid antibodies are identified in the mother, clots can be prevented through treatment with heparin, coumadin or other anti-clotting medications.   

If the baby is born, it is more likely to suffer from heart block - an abnormal heart rhythm that may require the baby to receive a pacemaker shortly after birth.  This is quite rare, but a known complication of pregnancy in women with Sjogren's.  

The baby could also be born with a form of Lupus that is believed to be temporary - visible signs may include the butterfly malar rash on the baby's face.  This is because the SS-A and SS-B antibodies associated with Sjogren's can transmit from mother to baby.

Any woman with an autoimmune disease considering pregnancy should speak with her rheumatologist beforehand, to identify any possible complications and make plans to deal with such situations in advance.

Sjogren's patients have a 44-fold increase in the risk of developing non-Hodgkin's lymphoma, compared with the general population.  If you understand the basics of the underlying Sjogren's disease process, which is that certain immune cells don't die off soon enough when they are supposed to (B-cells), them lymphoma makes sense, because lymphoma is a proliferation of too many B-cells sticking around for too long in the body.

So as you can see, Sjogren's is not just dry eyes and dry mouth.  For most Sjogren's patients, dry eyes and dry mouth are the least of their concerns!

My next post will be about Sjogren's treatments... stay tuned.


  1. My daughter has had positive markers for sjogren's for many years and though she has seen several of the most noted specialists for POTS no one has yet implicated the sjorgen's -- not sure why that is though she seems to have so many potential causes -- she has been on plaquenil for many years for the autoimmune issue. Have you had any other recommendations? Do you know how they make the actual determination that it is the sjogrens that is responsible? She has been seeing a rheumatologist for many years who feels that the sjorgens is not an issue --?
    I wish you all the best. I know how overwhelming this can be; research is really just starting and we have every reason to be hopeful that more effective treatment is not far away. God Bless.

  2. In response to stayingstrong's comment. I strongly encourage you to see some different doctors. I saw one rheumatologist who didn't even think to test me for Sjogren's, even though my autonomic neuropathy was already well documented with conclusive tests. I saw several neurologists who told me Sjogren's was a possible cause of autonomic neuropathy, but then they only tested the SS-A, SS-B antibodies for Sjogren's and when those came back negative, they told me I didn't have Sjogren's. It wasn't until I went through dozens of doctor's that I found one smart enough to figure this out. He told me that ONLY a lip biopsy was definitive for Sjogren's in some patients, and when I finally had a lip biopsy I was 100% positive.

    Here is a very important journal article about Sjogren's and autonomic (and other forms) of neuropathy you should share with your doctors:

    Here's a more informal article from Sjogren's World written by a doctor:

    While Plaquenil can assist with other aspects of Sjogren's Syndrome, it is not consider an effective treatment of the neurological manifestations of Sjogren's. Corticosteroids and IVIG are the drugs most often used for that. Neurological manifesations of Sjogren's are considered to be the more serious "extraglangular" manifestations of Sjogren's and should be treated more aggressivly that symptoms like dry mouth and dry eyes, which are usually just treated symptomatically. I am shceduled to begin IVIG infusions next week, and my doctors are confident that it will stop additional nerve damage, and likely reverse the nerve damage I already have, which in theory should reverse my POTS symptoms too.

    What kind of rheumatoligst thinks Sjogren's is not an issue? Does he think she doesn't have Sjogren's, or does he think it's not a big deal? That is concerning. What markers were positive? Has she had a lip biopsy?

    Feel free to contact me directly at laurenstiles at gmail. I would love to talk to another Sjogren's POTSie. We are a rare breed!

    1. Hi,I know it's been about 2 years since this was posted but I have a few questions. I am newly diagnosed with Sjogren's and my Rheumy suggested my tachycardia and elevated blood pressure,as well as shortness of breath could be POTS. I saw a cardiologist who determined there was no cardiac problem and merely said there's not a lot we can do about POTS other than include more salt in your diet and keep your blood pressure a little above normal. He did say there was some autonomic dysfunction but since my heart rate only went up to 115 bpm within 90 seconds it was subclinical. I don't know about you but my problem is that the longer I am standing the higher my pulse rate goes. It goes up to 135 to 150 when I am up and standing still for about 5 minutes. I go back to my Rheumy in a couple of weeks and will see what he has to say about further testing. Do you have any suggestions as to questions I might ask him? I am seronegative for Sjogren's so far but positive ANA and lip biopsy. We'll see what happens with those markers with the next round of testing for my 4 month follow up. And finally, how long did it take for the Plaquenil to kick in fully? I can only take 200 mgs a day because of side effects but it seems to have made a bit of a difference already in that I have more "good" days. As long as the POTS cooperates that is. Lol. Thanks, Katherine

  3. HI! I read your post and I have had almost all of these symptoms throughout the course of my disease. Right now I am dealing with horrible flare up symptoms. I am so glad I came across your blog cause I've been wanting to be able to relate to somebody who has similiar medical history like me. I think that I am a strong candidate for having an autoimmune disease but my doctor hasn't really been wanting to deal with it much. They know I have an undifferentiated autoimmune disease but that is as far as it goes. How did you first find out you had sjrogren's? Is your doctor on top of your treatment and everything? Thank you so much for writing this blog! I will definitely keep following! :)

  4. Hi. I am new to this. Diagnosed w sjorgens 5 months ago. Have battled tachycardia pots like symptoms for over two years. Having internal vibrations in head and body constant. Fatigue joint pain tachycardia worsens off and on face and extremity numbness tingling. Rhuemy put me on plaquenil.
    Sounds like I need more of an aggressive treatment. Ivig and steroids. Did this help your pots symptoms as well. What other Meds have u tried?

    Thanks so much for the valuable information :).

  5. Thank you for posting this. This is very good information that I wish everyone understood. -Debbie

  6. Just found your blog and wanted to thank you for it. I am a 56year old English lady and have just been diagnosed with pots after 2 years of different doctors and tests. After reading yor pages I had a good discussion with the doctor about the cause of my pots. He thinks it may be autoimmune and will have me tested for Sjogrens and antiphospholipid syndromes as he thinks it could be either of those. So thank you for posting your thoughts and information it has been a great help.