Another Crossover Illness with POTS--Sjogren's Syndrome

Sjogren's Syndrome-- Is an autoimmune disease named for Henrik Sjögren, a Swedish ophthalmologist. It is mostly known for causing extremely dry eyes and mouth. But it affects MANY other parts of the body because it affects the exocrine glands. The exocrine glands secrete their products to the outside of the body. These glands include the lacrimal and salivary glands and also glands in the stomach, intestines, respiratory tract, the skin, vagina as well as the pancreas and prostate. If their function is impaired it will affect the corresponding organs.

There are three types of Sjögren's syndrome. Primary Sjögren's syndrome occurs by itself, with no other associated disorders. Secondary Sjögren's syndrome occurs along with other autoimmune disorders, like systemic lupus erythematosus, rheumatoid arthritis, scleroderma, vasculitis, or polymyositis. If  the disorder only involves the eyes, it is called sicca complex.

About nine times as many women as men suffer from Sjögren's syndrome. Although most patients are diagnosed when they are between 40 and 55, SS affects all age groups. Thirty percent of people with other autoimmune diseases like Systemic Lupus Erythematosus and rheumatoid arthritis, also have SS. And a large percentage of SS patients also have POTS, and/or OT.

 Between 0.1% and 3% of the population in the United States have SS and it is reflected in all ethnicities. According to the American College of Rheumatology, between 1 million and 4 million Americans have Sjögren's syndrome.

Exactly what causes Sjogren's syndrome has not been determined. There are several possibilities. It runs in some families, indicating a genetic factor. The fact that more women than men have it points toward hormonal influences. And there are also viral factors. The immune system may be activated when the patient contracts a virus and then it becomes overactive and  mistakes areas of the body that produce moisture. In 2004 a group of Greek researchers presented evidence that a coxsackievirus may be the disease organism that triggers SS.

Most frequently SS causes dryness in the salivary glands, causing an extremely dry mouth. This is called xerostomia. This leads to difficulty swallowing and speaking. Because there is a reduction of saliva to help keep the teeth clean,  cavities and gum disease are also a prominent result. Saliva is produced mostly by the parotid glands. They lie over the jaw bones and behind the cheeks and in front of the ears. They frequently become enlarged in patients with SS.

The extremely dry eyes found in SS is caused by the lacrimal glands which produce tears, being slowly destroyed. This results in itching, burning, redness, sensitivity to light and thickened secretions that gather at the corners of the eyes. In some cases, the cornea develops ulcerations.
Other moisture producing glands in other areas of the body can cause many other symptoms. In the respiratory tract, dry mucous membranes in the nose can cause sinus infections and nosebleeds, not to mention discomfort. There can also be hoarseness, bronchitis, pneumonia, and ear infections. It can also cause inflammation and damage to alveoli(air sacs) in the lungs, resulting in scarring and breathing difficulty.  Patients can have very uncomfortable vaginal dryness resulting in yeast  infections and urinary symptoms.

I found out the hard way that the pancreas and gallbladder can be involved. This is due to slowed production of digestive enzymes and the pancreas itself works similarly to salivary glands and in conjunction with them. When you chew food a digestive enzyme is released in your mouth and another one is released by your pancreas. But if you think about it, it makes perfect sense. They are liquid. If they aren't functioning, then there can be problems digesting food  and absorbing nutrients like B12 which causes pernicious anemia. If you also have something like lupus, you are already at risk of anemia because of increased cell apoptosis.The role of apoptosis in systemic lupus erythematosus My gallbladder was necrotic and the cystic duct was gangrenous and the cystic artery was thrombosed. During my surgery, I lost 10 ml of blood. If I had lost anymore they would have had to do a blood transfusion. The whole thing ended up causing pancreatitis.

Liver diseases have been found in about a quarter of patients with Sjögren's syndrome. These can include Primary sclerosing cholangitis (PSC), a chronic, or long-term, disease that slowly damages the bile ducts, Primary biliary cirrhosis (PBC),autoimmune liver disease,  and in some areas of the world, Hepatitis C. 


There are also problems with the esophagus and the rest of the digestive tract due to decreased secretions. One very obvious problem with SS and digestion is constipation. If there aren't enough secretions in the intestines and not enough moisture, you are going to be constipated. Medications to reduce reflux are frequently prescribed and many patients have strictures and other problems with the esophagus and swallowing called dysphagia. I have had strictures, and esophagheal spasms, GERD and I have Barrett's Esophagus.  Esophageal motor function in primary Sjögren's syndrome: correlation with dysphagia and xerostomia  Dysphagia and other manifestations of oesophageal involvement in the musculoskeletal diseases Mayo Clinic: Dysphagia  You can also have abnormal liver function tests, chronic active autoimmune hepatitis and primary biliary cirrhosis.

Here are a few other links for some of the digestive problems with SS. gastrointestinal-effects-of-sjogrens   Sjögren's syndrome - information for patients and professionals by Dr. Joop P van de Merwe

 Kidney problems such as glomerulonephritis(inflammation of the glomerulus filters in the kidney) are also not unusual. This will cause, edema or swelling, blood in the urine and reduced urine output. Again this makes perfect sense because your kidneys are what maintain the balance of fluids in your entire body. Patients also have symptoms that have nothing to do with their glands including, fatigue, muscle aches and pains, fevers and joint pain.  Sixty-one percent  of patients with primary Sjögren's syndrome have severe urological symptoms compared with 40% of control patients
with osteoarthritis.  One of these problems is bladder irritability associated with urinary urgency and overactive bladder.  The overactive bladder associated with Sjögren's syndrome is an autoantibody-mediated disorder of the autonomic nervous system,which may be part of a wider spectrum of cholinergic hyperresponsiveness. This is basically the autonomic nervous system or in other words, dysautonomia. Patients  can also have interstitial cystitis.  This is inflammation in the interstitium of the bladder. Symptoms include pain, pressure or discomfort in or around the bladder, a persistent urge to urinate and frequent urination both in the daytime and at night. When the bladder is full, the pain is worse. There isn't any urinary tract infection, though. There are other names for it such as painful bladder syndrome(PBS) and hypersensitive bladder. Kidney stones are a problem as well, and those are quite painful and urinary tract infections are frequent as well. I am plagued with both. 
SS patients also have skin problems such as dry patches or overall dry skin, vasculitis. Cutaneous B-cell lymphoma(generally easier to think of as just lymphoma) is something that SS patients are at increased risk of. 

 As previously mentioned, Sjogren's can affect the bladder due to dysautonomia. Sjogren's syndrome can also cause damage to the nervous system which causes peripheral neuropathy, which is damage to the nerves in the arms and legs. This results in pain, numbness, and weakness. When it causes cranial neuropathy, it can cause headaches  and loss of taste and smell. And the parasympathetic system can be affected, which causes POTS and OT. Dysautonomia International: Underlying Causes of Dysautonomia It can also cause digestive motility problems because those are part of the autonomic nervous system via the parasympathetic nervous system. It results in yet another reason for difficulty swallowing, nausea, vomiting, and constipation. This can be exacerbated by inflammation. Diarrhea can also be a problem. If your pancreas isn't working properly and releasing digestive enzymes, then you won't be able to absorb fats and will have oily diarrhea.
 About 1/3 of Sjogen's patients have some form of thyroid disorder.An underactive thyroid gland can cause constipation and fatigue.  Thyroid problems can be treated, but they are often underdiagnosed. 

The all over pain and fatigue that often accompanies SS can be very debilitating as if you have the flu and it won't go away. 

And all of this can be secondary to other autoimmune diseases and the symptoms that go along with them. Antiphospholipid Syndrome is an autoimmune disease that causes bleeding and clotting problems, and apart from the problems this can cause the patient themselves, it can also put them at risk during pregnancies and cause miscarriage. Babies born to mothers with SS can have a condition called fetal heart block. Because of the association with these other conditions, such as lupus and rheumatoid arthritis, those patients will also be at risk for developing POTS and OT. 

Sjogren's syndrome has no cure. The symptoms can be treated in order to relieve some of the discomforts and try to avoid complications. Artificial tears are available over the counter and there are also prescription eye drops but those have an increased risk of malignancy. Some patients need to use these every 30 minutes or so. There is also a procedure called punctal occlusions, where they seal off your tear ducts so you maintain more tears in your eyes. Keeping something, especially water close at hand in order to take frequent sips of will help some with dry mouth. Sugarless gum and other lozenges can also be helpful. There are also mouthwashes for dry mouth. Pilocarpine(Salagen) and cevimeline(Evoxac) are prescription medications to increase saliva and tears. In order to avoid tooth decay and gum disease, special attention to dental hygiene is necessary. There are gel lubricants to treat vaginal dryness. Humidifiers and avoiding cigarette smoke are a good idea. Over the counter lotions and moisturizers help the dry skin. Other complications of Sjogren's can be helped with steroids. And an anti-malarial drug called Hydroxychloroquine(Plaquenil) is also frequently prescribed. 

Diagnosis is made through tests of your eyes to see how much tears you produce as well as biopsies of the salivary glands and blood tests for specific autoimmune antigens. You can read here about how it is diagnosed. http://www.sjogrens.org/home/about-sjogrens/diagnosis

Recently, attention has been brought to the condition in the media because Venus Williams has it.

After you watch the Sjogren's Syndrome videos, don't forget to watch the POTS videos at the bottom of the blog. You have to scroll down for them.

Sjögren's Syndrome - CRASH! Medical Review Series

Sjogren's: A Place to Begin - Part 1: What is Sjogren's Syndrome?

Sjogren's: A Place to Begin - Part 2: Management & Treatment of 
Sjogren's

Sjogren's: A Place to Begin - Part 3: Personal Experience: Estrella

Sjogren's: A Place to Begin - Part 4: Personal Experience: Cathy

Tips for Sjogren's Syndrome- Now With Captions!

Let's Talk About Pain: Sjogren's Syndrome & Rhumatoid Arthritis

Some More Videos On Kidney Function and Fluid Control In The Body and The Relationship To Blood Pressure

This is a series of videos that go together. They are on sodium and potassium disorders in the body, but they also explain kidney function, because sodium and potassium are directly related to fluid in the body, and therefore blood pressure.

After you watch the Kidney Function videos, don't forget to watch the POTS videos at the bottom of the blog. You have to scroll down for them.



Sodium and Potassium Metabolism (Renin, Angiotensin, Aldosterone, and ADH)

Hyponatremia

Hypernatremia

Hypernatremia - Examples

Hypokalemia

Hyperkalemia

Some Informative Videos On: Orthostatic Hypertension and Baroreceptors and how your kidneys control Blood Pressure

I found a few informative videos on Orthostatic Hypertension and Baroreceptors and how blood pressure and Heart Rate and Autonomic Nervous System work. Some of them are a little tedious, but if you want to understand how blood pressure regulation works, I recommend them. Watch them in order, and because they repeat some of the information, things start to click.  I am probably going to have to watch them more than once to retain the information. Brain Fog is a bitch. 

After you watch the Orthostatic Hypertension videos, don't forget to watch the POTS videos at the bottom of the blog. You have to scroll down for them.

Blood pressure regulation - Baroreceptors

Part I - Regulation of Blood Pressure (Hormones)

Part II - Regulation of Blood Pressure (Hormones)

Renin Angiotensin Aldosterone System (RAAS) - Short and sweet

General overview of the RAAS system - Cells and hormones

Renin production in the kidneys

FUNCTION OF THE NEPHRON made easy!!

Reabsorption in the Nephron

The Excretory System: From Your Heart to the Toilet - CrashCourse Biology #29

Diagrams of The Sympathetic and Parasympathetic Nervous Systems

Poor Man's Tilt Table Testing- How To

Neurogenic Orthostatic Hypotension- Another Category for Orthostatic Hypotension

Things That Help or Hurt When You Have POTS or Orthostatic Hypotension And Contributing Factors

• Sitting or standing for long periods (shop in off-peak hours. Move your legs around)
• Warm environments (hot showers, saunas, hot tubs)
• Sodium/salt depletion 
• Prolonged bed rest
• Varicose veins
• High carbohydrate meals
• Diuretics, vasodilators, alpha-blockers
• Alcohol

  This is because they result in venous pooling and hypovolemia. 

• Stress
• Excercise
• Pain
• Hypoglycemia (low blood sugar)
• Albuterol
• Epinephrine

 They will increase catecholamines.

Some helpful things are:

• Raising the head of your bed
• Compression Garments i.e. support hose, body shapers/girdles. abdominal binders
• Waist high are better than thigh high which are in turn better than knee high 
• Standing with legs crossed
• squatting
• sitting knees to chest
• leaning forward when you are sitting
• elevating your knees when sitting (try using a foot rest)
• clenching your fists when you are standing up

All of these use your own muscles to pump blood. 

• Drink a minimum of 2 liters a day/0.52 gallons/67 ounces
• Drink at least every 2 hours
• Avoid sleeping more than 12 hours a day because you can't drink in your sleep
• Increase your salt according to your taste and add salt tablets

Even though you have exercise intolerance, some exercise is good. Being inactive is bad

• Avoid excessive bed rest/sleeping
• Start excise slowly and gradually increase
• Recumbent exercise bikes or lying on your back are good 
• Swimming
• Manual forms of Physical Therapy can be helpful

You need to be treated for any conditions that contribute to your orthostatic intolerance;

• food allergies
• anxiety
• depression
• pelvic vein incompetence
• EDS/JHS  ( Ehlers-Danlos Syndrome/Joint Hypermobility Syndrome)
• Chiari 1 malformations
• stenosis of the cervical spine
• Migraines
• movement restrictions
• Infection
• allergies/asthma because inhalants make you worse

Medications: Can be used alone or in combinations

• Vasoconstrictors: Midodrine, dexodrine, methylphenidate, SSRIs, SNRIs, aescin(horse chestnut seed extract), L-DOPS(Droxidopa) is being used in trials.
• volume expanders: Sodium consumption or IVs, fludrocortisone/Florinef, clonidine,
• catecholamine release inhibitors:beta blockers, disopyramide, SSRI's, ACE inhibitors
• Other treatments include but are not limited to:  pyridostigmine bromide

Common Findings in (POTS) Postural Orthostatic Tachycardia Syndrome

• More common in women than in men
• It often comes on slowly  or after infection, surgery, or trauma
• Symptoms are often disabling
• People suffer from: fatigue, exercise intolerance, palpitations
• Some of the Pathophysiologies are:

1.  Hyperadrenergic adrenergic/neuropathic forms
2.  Some classify into low, normal and high-flow POTS
3.  Sub categories include hypovolemia, elevated PRA/Aldosterone ratios, AChR ab positive,         NET deficiency

In both POTS and Orthostatic Hypotension, there is often acrocyanosis. This means that hands, lower legs and feet often have a purplish appearance from peripheral cyanosis. 

Symptoms of Orthostatic Hypotension

• Lightheadedness
• Syncope
• Diminished Concentration
• Headache
• Blurred vision
• Fatigue
• Exercise intolerance
• Dyspnea
• Chest Discomfort
• Palpitations
• Tremulousness
• Anxiety
• Nausea
• Nocturia

Common Things Found In Orthostatic Hypotension

• It is the most common cause of recurring syncope
• It is more common in women, young people, and people with low-normal to low BP.
• Common following infection
• Family members are also often affected with the condition
• Findings from physical and lab tests are frequently normal
• Hypotension or low BP goes undetected unless orthostatic stress is prolonged
• Fatigue lasts for hours after syncope

Synonyms for Orthostatic Hypotension

• Neurally Mediated Hypotension
• Vasovagal Syncope
• Neurocardiogenic Syncope
• Vasopressor Syncope
• Neurally Mediated Syncope

Orthostatic HYPERtension: What Is That?

I recently had a short stay in the hospital for low blood pressure and a slow heart rate(also called Bradycardia). I had a few EKG's, an Echocardiogram, and a Stress Test. The doctors took me off of my beta blocker that I have been on for years. When my blood pressure leveled out, they sent me home.

My doctor was out of town at the time and not expected to be back for a few weeks. A few days after I got home, I started having dizzy spells (Syncope). So, I started checking my blood pressure and it was high. I decided I didn't want to go back to the hospital and would wait it out and see what my doctor said when she got back. Since I had gone from really low BP and heart rate to having high BP and HR, I decided to look up how to do a "Poor Man's Tilt Table Test". You can read more on how to do this here. And there is a link to the right on what a tilt table test is.

I had some help doing it, but we did three of them. All three of them caused my BP and HR to raise over the requisite 30 beats above the resting BP and HR.

I knew what Orthostatic Hypotension was. It is sometimes also called Neurally Mediated Hypotension.But with it, your blood pressure drops 25 mmHg, without the increase in HR that I was having. When my husband was diagnosed, they didn't separate out Orthostatic Hypotension from POTS. And depending on who was talking to us, they were using the two terms interchangeably. Now they are two distinct diagnoses, although you can have both.

 I was pretty concerned because I had never heard of BP going up to the degree mine was when I stood up.  So, I did a google search.

I found out that there is a subclass of orthostatic intolerance that causes this called ORTHOSTATIC HYPERTENSION. This is what I could find out about it. There wasn't much information available.

In general, it is simply when your BP rises upon standing. Orthostatic hypertension was defined as either an increase in DBP (diastolic)  from <90 mmHg to ≥90 mmHg or an increase in SBP(systolic) from <140 mmHg to ≥140 mmHg.

Studies have shown that people who have Mast Cell Activation Syndrome and POTS sometimes have Orthostatic Hypertension. People with baroreflex failure also have bouts of it. Another rare condition  called norepinephrine transporter deficiency can also cause this increase in blood pressure on standing. And pheochromocytoma can also cause it. And finally, diabetes can cause it. Diabetics are prone to having neuropathy (nerve damage), and it is likely that neuropathy has some connection to Orthostatic Hypertension.

Patients usually have venous pooling in the lower legs. This means that a large amount of blood collects in your lower legs when you stand up. This causes a decrease in the cardiac output of your heart. That in turn, causes your sympathetic nervous system to become activated. And then your arteries become severely constricted. This is similar to the POTS occurs. POTS happens because of dysautonomia in the lower legs which results in venous pooling. But the difference is that POTS patients don't always have symptoms of Orthostatic Hypertension.

Different studies have used different criteria for diagnosing Orthostatic Hypertension.
One study used the following:  ≥20 mmHg increase in SBP upon assuming an upright posture ( head-up tilting to 70 degrees) from the supine position.

Generally, this result needs to be reproducible because people with conditions like essential hypertension (regular high blood pressure) or diabetes have more variation in blood pressure readings than otherwise healthy people.

Aside from the rise in BP when you stand there are other tests that need to be run in order to diagnose underlying causes. Your doctors need to know if there is something that needs to be corrected with surgery, or if you have baroflex failure.

To treat it, the most obvious line of treatment is to treat your high blood pressure, if you have it. Activation of the sympathetic nervous system should be controlled with things like alpha-1 adrenergic receptor antagonists such as prazosin, or central alpha-2 adrenergic receptor agonists such as clonidine.

More on this subject can be found here: Orthostatic Hypertension: When Pressor Reflexes Overcompensate