What is Raynaud's (ray-NOHDZ) disease?

What is Raynaud's (ray-NOHDZ) disease?

It is a condition that causes some of the areas of your body, usually but not always the extremities, such as your fingers and toes — to feel numb and cold when they become cold. Stress can also cause symptoms.   When you have Raynaud's disease the smaller arteries which supply blood to your skin narrow, decreasing the blood flow to affected areas. This is called vasospasm.

More women than men have Raynaud's disease. It is also called Raynaud's phenomenon or syndrome. People who have it usually just say, “Raynaud's” without the other descriptive words. People who live in colder climates are more likely to have it.

If you have Raynaud's, your treatment is largely dependent upon how severe it is and also any other conditions you may have. It isn't usually life threatening, but it does affect how you live on a day to day basis.

Simple things can bring on an attack, like digging around in your freezer for that roast you know is in there somewhere. It doesn't take long for your hands to turn colors and hurt. And it gets worse when you take your hands out of the freezer and the circulation comes back into them. Other things like going out into the cold or putting your hands into cold water can cause the same symptoms.

Some of the symptoms include:

Cold fingers or toes
Color changes in your skin in response to cold or stress
Numb, prickly feeling or stinging pain upon warming or stress relief

Your fingers or toes get cold and then they turn white. Then they progress to blue and start to feel numb and cold. And then when you seek to warm them up, and the circulation starts to come back into them, they become painful, with throbbing and tingling and they turn red. Some people like me don't have all three color changes. I usually don't turn blue. But that may be because I avoid getting my hands and feet cold. They may never get cold enough to cause the change from white to blue. But I have had them do that years ago. It can take as much as 15 minutes for the blood flow in an affected area to return to normal.

There are other areas of the body besides the fingers and toes that can be affected by Raynaud's. Your ears, lips, and nose can be affected.  Raynaud's can even affect the internal organs. And even more embarrassing areas like your nipples and your rear end. Yes, now you can tell your husband that “turkey butt” is a medical condition and you can't help it.



I purposefully chose photographs that don't look too severe. Not everyone looks like they are ready for amputation when they have an attack of Raynaud's and if you look at the severe cases you might not recognize it in yourself. If you have bad discoloration you can google it and come up with photos.

The exact cause of Raynaud's hasn't been determined yet. Something causes the blood vessels in the hands and feet to overreact to cold temperatures or stress. This overreaction is called vasospam. During vasospasm the arteries that supply blood to your fingers and toes spasm due to cold exposure or stress and narrow which causes a decrease in the blood supply. This eventually causes the arteries to thicken which further limits the blood flow.

Raynaud's phenomena in real time.



There are two classifications of Raynaud's.

PRIMARY RAYNAUD'S
It is also called Raynaud's disease and it's usually isn't caused by some other medical condition. It usually begins earlier in life than Secondary Raynaud's between 15-30 years of age. There seems to be a genetic predisposition to Primary Raynaud's. Approximately 1/3 of patients with Primary Raynaud's have a parent, sibling or child who also has it the disorder.

SECONDARY RAYNAUD'S
It is also called Raynaud's phenomenon and is less common than Primary Raynaud's. and it usually is caused by something else. It is usually more serious. Onset is usually around the age of 40.
There are two main types of the condition.

Things that cause Secondary Raynaud's are Connective tissue diseases like Scleroderma, Rheumatoid Arthritis, Sjogren's syndrome, and Lupus. Scleroderma can cause a hardening or thickening of the blood vessels. Sjogren's, Lupus and RA cause inflammation of the nerves that cause the blood vessels to constrict. Up to 1/3 of Lupus patients have Raynaud's. And 15-30% of Sjogren's patients have Raynaud's. I have both Lupus and Sjogren's so it was probably inevitable that I would have Raynaud's.

Hardening of the arteries also called atherosclerosis and high blood pressure in the lungs called primary pulmonary hypertension also cause Raynaud's. These things can be exacerbated by smoking because it causes the blood vessels to constrict.

If you have Carpel tunnel syndrome causes numbness and pain in your hands due to pressure on the nerves. This can cause your hands to be susceptible to temperature changes and lead to Raynaud's. Operating tools which vibrate can cause you to develop Secondary Raynaud's.

Raynaud's disease is also associated with dysautonomia. With regard to Postural Orthostatic Tachycardia, Neurocardiogenic Syncope and Orthostatic Hypotension, an interview on Dysautonomia International had this to say:  “We do not know how many POTS, NCS or OH patients have APS, but Dysautonomia International recently funded a research project designed by Dr. Svetlana Blitshteyn to try to shed some light on the topic of autoimmune markers and autoimmune conditions in patients with POTS.  Dysautonomia International will make an announcement when Dr. Blitshetyn’s study results are released.” And in the article, it says that Raynaud's disease is a symptom of Antiphospholipid Syndrome. So you can say that Raynaud's is associated with dysautonomia.What Dysautonomia Patients Should Know About Antiphospholipid Syndrome Postural Orthostatic Tachycardia SyndromeA Dermatologic Perspective and Successful Treatment with Losartan

That's not too surprising. People with dysautonomia and POTS have problems regulating temperature in general and blood flow problems because of inadequate pressure in the blood vessels. And POTS, like Raynaud's disease, is associated with autoimmune diseases.

Causes

Beta blockers, migraine medications, ADD/HD medication, and cold medications can all cause Raynaud's or make it worse because they cause constriction of the blood vessels.

There are also some chemicals that can cause Secondary Raynaud's.

It can become severe enough that you need to seek medical help. You definitely need to see a doctor if you get a sore on one of the areas affected so that you don't end up with an infection and lose an appendage.

Some people have a permanent reduction or blood flow which causes their fingers and toes to become deformed. If a complete blockage of an artery occurs, you can get skin ulcerations and ultimately gangrene, which is why it is important to see a doctor if you get a sore on an affected area to avoid having an amputation.

Treatments

There are things you can do for Raynaud's. Wearing warm clothes, socks and gloves in cold weather are essential. Ear muffs and masks and scarves to keep your nose warm are also good ideas. Some people wear the socks and gloves when they are sleeping. And the gloves come in handy when you have to get into the freezer, but I personally never remember to do it. Avoid getting cold in the first place. Smoking is a no-no. Avoid stress because it can bring on an attack. And exercise increases circulation.

If you get cold, go inside and warm up. Two things that you should do are almost instinctive for people to do. Wiggling your fingers and toes and rubbing them to get them warm. You can also put your hands in your armpits and swing your arms to increase blood flow. You can run warm water over them too. Just don't get the water too hot.

Medications

And there are medications that help.

Calcium Channel Blockers relax the small blood vessels which help avoid the vasospasms. Common drugs in this class are Procardia, Norvasc, and Verapamil and Nifedipine.

Alpha blockers like prazosin and Cardura work by counteracting noradrenaline also called norepinephrine. This hormone causes blood vessels to constrict.

Vasodilators Nitroglycerin works as a vasodilator and comes in a cream and will facilitate the healing of ulcers. Losartan, which is a high blood pressure medication, and Viagra, as well as antidepressants like fluoxetine, can help treat Raynaud's.

Fish oil has fatty acids in that are supposed to make you less susceptible to cold.  Studies have shown that it didn't help people who have Secondary Raynaud's. You have to be careful when taking high doses of fish oil because it acts as a blood thing and increases your risk of bleeding, particularly if you take blood thinners, like as warfarin (Coumadin), clopidogrel (Plavix), or aspirin. Discuss it with your doctor.

Gingko is also supposed to be helpful by opening up the blood vessels. One study showed that Raynaud's patients had less pain if they took 160 mg of ginkgo per day. DO NOT take ginkgo if you have a history of seizures. Ginkgo can also increase risk of bleeding, especially if you take blood thinners. Talk to your doctor.

Evening primrose oil also contains a  type of fatty acid which keeps your body from making chemicals that narrow blood vessels.  Studies have shown that taking Evening primrose oil will lessen the severity of attacks as well as the frequency. EPO can make seizures worse in people who already have them and they shouldn't take it. And just like fish oil, there is a risk of bleeding with it.

There is a form of vitamin B3(niacin) called Inositol hexaniacinate. It may reduce the number of Raynaud's attacks. But it requires high doses and needs to be monitored by a doctor.

Taking Magnesium supplements opens the blood vessels, but there haven't been any studies to show if it works. Some people get diarrhea from taking it, so take it with a meal to help avoid this problem. It can also interfere with some medications like high blood pressure medication and antibiotics, so your doctor needs to be aware if you are taking it.

Biofeedback to lessen the effects of stress on your body may help lessen the frequency of attacks. Accupuncture is another treatment option that helps by improving blood flow to the affected areas. There is also a specific kind of biofeedback called Thermal Biofeedback that studies have shown works.

Surgery and injections

When lifestyle changes and medication don't help enough, sometimes things like surgery and injections of chemicals are the next line of treatment.

There are nerves in your feet and hands that control vasoconstriction and dilation. There is a surgery called a sympathectomy, in which these nerves around your blood vessels in your hands or feet are cut to stop the over-constriction. For some people it is helpful.

Botox injections can also block the sympathetic nerves and block the over-constricion.

For more information visit this link for a guide to Raynaud's
Raynaud’s Guide: The Cold Facts on Raynaud’s


These are links to my sources:

http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/definition/con-20022916

http://www.umm.edu/health/medical/altmed/condition/raynauds-phenomenon

Thermal Biofeedback

Biofeedback Therapy For Raynaud’s Disease Symptoms—raynauds.org

About Raynaud's Disease—Lupus.org

Our Top 5 Tips for Raynaud's Syndrome

Cold Weather & Raynaud’s Phenomenon

Raynaud’s Phenomenon—Rheumatology.org

http://www.rheumatic.org/primer.htm

Raynaud's Awareness

Pathophysiology Raynaud's Disease  

What Is Dysautonomia?

What IS Dysautonomia?

Autonomic Neuropathy Treatment & Management

Autonomic Neuropathy Treatment & Management--Medical Paper

If You go to the above link and create a sign in password this is a great medical paper/article. 

Dysautonomia International’s Videos-- A Whole Bunch Of Them On Vimeo

There are pages and pages of these videos. I have a lot of watching to do. I've put some of the more interesting ones up here. 

https://vimeo.com/dysautonomia/videos

Autoimmunity in POTS - Dr. David Kem

Research Updates: MCAS, Gastroparesis & Sjogren’s

Mast Cell Activation Syndrome & Dysautonomia - Dr. Lawrence Afrin

Post-Concussion Dysautonomia - Dr. Glen Cook

POTS Non-Pharmacologic Therapy

Gadgets, Gizmos & The New World of Syncope - Dr. Blair Grubb

Sudden Steep Drop in Blood Pressure on Standing From Lying Down May Predict Atrial Fibrillation Years Later

http://www.hopkinsmedicine.org/news/media/releases/sudden_steep_drop_in_blood_pressure_on_standing_from_lying_down_may_predict_atrial_fibrillation_years_later

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 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

Orthostatic HYPERtension As Opposed to HYPOtension

There is a difference between Orthostatic Hypertension and Orthostatic Hypotension. It seems to be more unusual and there isn't much information to be found. I did, however, find some since that seems to be more in line with my personal symptoms. Here are the links to the PDF at two different sites. 
http://www.medscape.com/viewarticle/543590 http://www.nature.com/nrneph/journal/v2/n8/full/ncpneph0228.htmlAt medscape, you have to create a username. But it is free and worth it because they have many medical articles that you can't find elsewhere.  



This is another one, AHA Journals: Orthostatic Hypertension Due to Vascular Adrenergic Hypersensitivity

Orthostatic Hypertension in Patients With Type 2 Diabetes

AHA Journals: Relationship Between Extreme Dippers and Orthostatic Hypertension in Elderly Hypertensive Patients

I am going to try and give a brief overview (well as brief as possible). 
This is a direct quote from the first article: "Orthostatic hypertension—a rise in blood pressure upon assuming upright posture—is an underappreciated and understudied clinical phenomenon. There is currently no widely agreed-upon definition of clinical orthostatic hypertension, the current definitions being operational within the context of particular studies. The underlying pathophysiology is thought to involve activation of the sympathetic nervous system, but the actual etiology is poorly understood. Orthostatic hypertension is observed in association with a variety of other clinical conditions, including essential hypertension, dysautonomias, and type 2 diabetes mellitus. Orthostatic hypertension has been associated with increased occurrence of silent cerebrovascular ischemia and possibly with neuropathy in type 2 diabetes. So, appreciation of the true incidence of orthostatic hypertension, elucidation of the underlying pathophysiology, and an understanding of potentially effective treatment approaches and their associated risks and benefits might all have major clinical significance. Orthostatic hypertension is an aspect of hypertension that is in need of further focused investigation."

Ordinarily, when you change from lying down or sitting up to a standing position, your blood pressure will drop slightly. This is because of  "a redistribution of blood volume into the lower abdomen, buttocks, and legs under the influence of gravity".

For most people, the change is very slight and systems in your body quickly engage and compensate for the change in positions. Usually, baroflex receptors cause some of your arteries like the ones in your neck to stretch so that you can get more blood flow to your brain. And your body senses a decrease in BP in your chest and abdomen and your sympathetic nervous system kicks in and increases activity and there is also a corresponding decrease in the parasympathetic nervous system. Basically, this means that hormones in your body regulate your blood pressure to compensate for your change in position from prone to sitting and from sitting to standing.

Because your blood plasma volume decreases, your systolic blood pressure increases and your diastolic blood pressure drops slightly, as well as having a slight rise in heart rate. There is also an increase in norepinephrine, epinephrine, active plasma renin, aldosterone, and vasopressin. Your sodium reabsorption increases and your potassium excretion are increased by your  kidneys. All of this is controlled by your sympathetic and parasympathetic nervous system, and in turn controlled by the baroflex response.

I have other posts here about orthostatic HYPOtension. That is when your BP drops 20/10 mmHg when you stand up. You also have of cerebral hypoperfusion, including dizziness or lightheadedness, visual changes, discomfort in the head and neck, fatigue, and frank syncope.


The first article above says that POTS frequently presents with no drop in blood pressure, or with a mild orthostatic HYPERtension.

Again, orthostatic HYPERtension is when your BP raises when you stand up. In order for there to be a diagnosis of Orthostatic HYPERtension, there needs to be an increase in systolic BP of 20 mmHg. How this plays out for me personally is very confusing. For instance, sometimes, my BP will raise by the required amount when I sit up from lying down, and then it will gradually drop as I am standing. Other times it has raised dramatically, even higher than the required amount. I do not have high hopes of getting a diagnosis, as I have been hospitalized recently for both low blood pressure and high blood pressure. I think at the very least I have some severe dysregulation.

 My late husband and father both had classic orthostatic HYPOtension and POTS. I would truly like to have a greater understanding of these conditions because I have children and grandchildren who could potentially develop them. Most people think that it any of these disorders are just a condition that you have to live with and they are just annoying. 

According to the article: " orthostatic hypertension might be a symptom of another treatable condition, such as pheochromocytoma or mast-cell activation disorder in the context of POTS.And "orthostatic hypertension resulting from any number of causes might be an important risk factor for silent cerebrovascular ischemia and infarct. 

Published studies from the Shimada laboratory indicate that, at least in populations of elderly Japanese people with essential hypertension, the incidence of silent cerebrovascular infarct detectable by MRI is higher in those patients who have clinically identifiable orthostatic hypertension."Some of these terms like pheochromocytoma are unfamiliar and confusing so I am going to link them to definitions when possible. I should have done that with my earlier posts. That one is basically a non-cancerous tumor on your adrenal gland.

These are the symptoms:

• High blood pressure
• Rapid or forceful heartbeat
• Profound sweating
• Severe headache
• Tremors
• Paleness in the face
• Shortness of breath
• Anxiety or sense of doom
• Abdominal pain
• Constipation
• Weight loss

For some people, the spells last 15-20 minutes and can happen several times a day. Some people's BP is normal between spells and for others it stays elevated.These are some of the triggers:

• Physical exertion
• Anxiety or stress
• Changes in body position
• Bowel movement
• Labor and delivery

Foods high in tyramine, a substance that affects blood pressure, also can trigger a spell. Tyramine is common in foods that are fermented, aged, pickled, cured, overripe or spoiled. 

These foods may include:

Some cheesesSome beers and wines

Dried or smoked meats

Avocados, bananas, and fava beans

Pickled fishSauerkraut or kimchi

Certain medications that can trigger asymptomatic spell include:

Decongestants

Monoamine oxidase inhibitors (MAOIs), such as phenelzine (Nardil), tranylcypromine (Parnate) and isocarboxazid (Marplan)

Stimulants, such as amphetamines or cocaine

It is unknown what causes these tumors. Your body uses the adrenaline/epinephrine and norepinephrine produced by your adrenal glands, in the fight or flight response. Basically, in a stress situation, your body uses adrenaline to raise your BP and norepinephrine to slow down things in your body temporarily, like if a bear attacks you, you don't need to be digesting supper at that moment, so that would slow down in order to shift resources to more important things like running away. A pheochromocytoma causes an excessive release of these hormones. Less common signs or symptoms may include:I found it interesting that the cells that can cause one of these tumors can be found in other areas of the body. When they do, they are called paragangliomas. They can be in your heart, head, neck, bladder, back wall of the abdomen and along the spine. I believe my husband may have had one on his spine. I know he had what they called a benign tumor of some sort there. But doctors didn't connect it to his condition, and I was ignorant. 

There are genetic conditions that predispose you to this condition.

These genetic conditions include the following:

• Multiple endocrine neoplasia, type II (MEN II) is a disorder resulting in tumors in more than one part of the body's hormone-producing (endocrine) system. The locations of other tumors associated with MEN II include the thyroid, parathyroid, lips, tongue and gastrointestinal tract.
• Von Hippel-Lindau disease can result in tumors at multiple sites, including the central nervous system, endocrine system, pancreas and kidneys.
• Neurofibromatosis 1 (NF1) results in multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.
• Hereditary paraganglioma syndromes are inherited disorders that result in either pheochromocytomas or paragangliomas.

Interestingly, I have been told I have benign cysts on my liver, my kidneys, and on my thyroid. I also have had problems with my pancreas. My father had a tumor on his pituitary gland. So there may be something here for me. 

Sometimes this requires the removal of the adrenal gland affected and it can be cancerous. I don't know what they do if the tumors are somewhere else in the body.

Mast cell activation syndrome is complicated. Mast cells, if you are familiar at all with them, are associated with allergic responses. They release histamines that cause itching and hives. They also have a role in healing the body and the immune system. Somehow they can also be paired on autonomic nerve fibers. Hence the association with dysautonomia.  

They can release up to 200 different kinds of chemicals, and they produce, histamines, prostaglandins, and leukotrienes. For some people, there may be a malfunction in this system.

People with mast cell problems can have abdominal pain, cramping, diarrhea, flushing, itching, wheezing, coughing, lightheadedness, brain fog,  and memory problems. Those symptoms can all be caused by other conditions. But there are some people who have these symptoms along with POTS.

There are tests for it. One is for tryptase, which is a protein that is made by mast cells. Elevated levels occur as a result of severe allergic reaction OR if your body makes too many of them, which is called mastocytosis. If you have too many, they will sometimes to a bone marrow biopsy. Mastocytosis is caused by a genetic mutation, that causes a cell to grow and divide uncontrolled. The treatment for it is different than for MCAS
Most people with POTS don't have elevated tryptase or mastocytosis. 

But they can have elevated histamines, prostaglandins and leukotrienes, which can be found in a 24 hour urine test. If they are elevated, then a diagnosis of MCAS, mast cell activation syndrome is usually made. 

Here are the criteria:

       Symptoms consistent with chronic/recurrent mast cell release

• a. Recurrent abdominal pain, diarrhea, flushing, itching, nasal congestion, coughing, chest tightness, wheezing, lightheadedness (usually a combination of some of these symptoms is present)
• Laboratory evidence of mast cell mediator (N-methyl histamine, prostaglandin D2 or 11-beta- prostaglandin F2 alpha, leukotriene E4 and others)
• Improvement in symptoms with the use of medications that block or treat elevations in these mediators

The article I got this information from on dysautonomia international's blog says that the labs need to be sent on ice or they might come back falsely normal. And that they should be done after a flare-up of symptoms is possible. They also say that they are not always 100% accurate and should be interpreted carefully. 

It says that if you have POTS or some other dysautonomia and also have some symptoms that seem allergic, you should ask your doctor about getting tested for MCAS. And it says that an allergist or immunologist is more likely to be familiar with MCAS.MCAS Mast Cell Activation Syndrome

Okay, back to the subject at hand, orthostatic HYPERtension.

Orthostatic hypertension

• Systolic blood pressure increases by at least 20 mmHg upon standing(note:other studies only require a 10 mmHg change)
• No change in diastolic blood pressure has been defined

That is different from Orthostatic HYPOtension, in which

• Systolic blood pressure decreases by at least 20 mmHg upon standing
• Diastolic blood pressure decreases by at least 10 mmHg upon standing

The older criteria for Orthostatic HYPERtension was a diastolic BP that was already 90mmHg and raised even higher when standing. While I am on beta blockers, mine tends to be just under that, at least part of the time. 

People with Orthostatic HYPERtension also have a decrease in cardiac output, more venous pooling in the lower extremities and higher plasma norepinephrine levels when they are standing. I have no idea how they measure that. I assume with blood tests for the norepinephrine and possibly measuring your legs for swelling or just observing changes in color. 

The theory is that the venous pooling in the lower body causes a decrease in cardiac output, which causes sympathetic nervous system response and an increase in diastolic BP. 

Around 11% of older people who already have high blood pressure or hypertension also have orthostatic HYPERtension. And they are more likely to have silent cerebrovascular infarct.

About the same amount of people have orthostatic HYPOtension and they are also at risk of a

silent cerebrovascular infarct. 

There are also people who are what they call an 'extreme dipper'. This means their systolic BP drops during the night. People who do this have a 72% likelihood of having orthostatic HYPERtension compared to the above 11%. They are also 53% more likely to have a silent cerebrovascular infarct. They are also more likely to have a regular stroke and don't recover as well. 

Both people with Orthostatic HYPERtension and HYPOtension are more likely to have lesions on the central nervous system that can be detected by MRI.

The medical article I read said that a recent study showed that people with POTS and MCAS, mast cell activation syndrome, 38% of them had Orthostatic HYPERtension. I feel it necessary here for clarity to define POTS.


Dysautonomia International says that it is "a heart rate increase of 30 beats per minute (bpm) or more, or over 120 bpm, within the first 10 minutes of standing, in the absence of orthostatic hypotension".

So we are discussing BP changes, but POTS is a heart rate change, that can sometimes be concurrent with the orthostatic changes, but is a separate diagnosis.

Some of the patients in the study had orthostatic HYPERtension that was persistent in an upright position or sometimes they had a hypertensive crisis. A hypertensive crisis is a BP that is 180/120 and can go as high as 240/140. They say that it hasn't been observed in patients with POTS bub without MCAS.


People with Baroflex Failure can also have orthostatic HYPERtension.

Baroreflex Failure is a rare disorder characterized by a change of blood pressure with episodes of severe hypertension (high blood pressure). There can be increased heart rate during stress and hypotension (low blood pressure) with normal or reduced heart rate during rest.

Symptoms may include:

• Headache
• Excessive sweating
• Extremely high or volatile blood pressure and heart rate with spikes in blood pressure in response to stress, with periods of normal or even low blood pressure during rest.
• Heart rate that does not respond to medications intended to improve it.

Causes of Baroreflex

Possible causes may include:

• Surgery and radiation for cancer of the throat
• Injury to the glossopharyngeal and vagus nerves (nerves involved in sensing blood pressure)
• Cell loss on both sides in the nuclei of the solitary tract (NTS, a column of cells located in the medulla) in the setting of a degenerative neurologic disease of the brain.
• For many patients, the cause is unknown.

I'm leaning towards this for my own problems because I have had both low blood pressure and high blood pressure and the rise in heart rate. Also, my urine test didn't indicate Pheochromocytoma.

The treatment for Baroreflex Failure is medication to control blood pressure and heart rate and to reduce stress. 

The purpose of Baroflexes is to buffer changes in arterial pressure so that excessive fluctuations of blood pressure are avoided. Baroreceptors are mechanoreptors in each carotid sinus brain stem and the aortic arch, and their function is to sense pressure changes caused by distention of the blood vessel walls send information about distention of the vessel wall by the glossopharyngeal nerves to the brain stem. There are baroceptors in the aortic arch and the large vessels of the thorax that transmit information through the vagal nerves to the same area of the brain stem. The blood volume in the thorax is sensed by low-pressure receptors that are linked to the vagal nerves and the brain stem. 

Problems in the baroceptors, the glossopharyngeal or the vagus nerves or the brain stem all could cause baroflex failure. Sometimes the terms baroflex failure and autonomic failure are used interchangeably. But usually, autonomic failure caused orthostatic HYPOtension and baroflex failure and the accompanying anatomical lesions usually cause volatile hypertension. 

Baroflex failure usually causes a loss of the buffering of blood pressure and  volatility of BP and heart rate. 

Baroflex failure can be  documented by the inability of infusions of pressor and depressor drugs to cause reflex bradycardia and tachycardia.25 mmHg or higher. If you have a heart rate drop of 25 mmHg it indicates baroflex control of heart rate.

They can put you in a supine position(laying down) and inject you with phenylephrine and increase the dose until your systolic BP raises 25 mmHg or higher. If you have a heart rate drop of 25 mmHg it indicates baroflex control of heart rate.

They have also injected people with nitroprusside until your systolic BP drops by 25mmHg and causes a change in HR. 

Apparently, there can be several causes of Baroflex failure. Some of the people in the study had previously had surgical damage of the glossopharyngeal nerve because of a neck injury. And some had radiation of the pharynx that caused problems and some other people had familial paraganglioma syndrome, which is a genetic problem that causes benign non-catecholamine producing tumors of the carotid body and glomus jugulare and glomus vagale. The tumors damage the glossopharyngeal and vagus nerves. One person also had cell loss in the nuclei of the solitary tracts of the brain stem that had been caused by a degenerative neurologic disorder. But some of the patients they coudn't find a cause for their baroflex failure. 

I certainly hope there is some other way to find out if you have it besides being injected with stuff.

After they did the injection part of the test, the patients were monitored. In contrast to the 24-hour urine test I had, during the study they measured catecholamines were measured every so often. And the BP was taken every 4 hours in supine and upright positions and anytime they had symptoms. 

They also did cold pressor tests. This is when they have you to put your right hand in a basin filled half with ice and half with water and keep it there for 1 minute. They measure your heart rate before and after. They did math tests by having people count backward from 200 by sevens. And they measured BP before and after this. And there is also a test called the isometric handgrip. 

They also gave patients propranolol and atropine in order to measure sympathetic and parasympathetic responses that control heart rate. They gave them Clonidine and monitored BP afterward to see how it affected reduced the sympathetic response. 

The patients BP would rise dramatically due to the cold pressor and math tests, which basically caused some mental stress.Some patients had increased nervousness or depression after they became ill. And it was worse with the patients with the worst BP elevation. When their BP was up, they had a sensation of flushing and were pale. They had palpitations and headache and sweating pheochromocytoma. But they had previously had this ruled out by urine and blood tests and radiographic tests and also by the improvement, or at least the absence of an increase, in hypertensive episodes during follow-up.

These patients had higher systolic BP than normal people. But they also had lower than normal BP at night, that "extreme dipper" thing. Their heart rates were also abnormal. Some of them had HR of 90 beats a minute, which could be from loss of parasympathetic control of the heart rate, caused by damage to the right vagal nerve. 

When they were measuring the norepinephrine during the tests, they were much higher than normal. And they raised in some patients  to more than 200 pg per milliliter (1.1 nmol per liter). Urinary excretion of epinephrine plus norepinephrine averaged 118 μg per 24 hours (697 nmol per 24 hours), more than twice normal (P = 0.015).

The cold pressor test caused a hypertensive paroxysm in some of the patients and lasted for a long time after they removed their hand from the cold water. That means their BP raised to a volatile level. 

They were able to show a greater drop in BP during bouts of hypertension with Clonidine, more so than when their BP was at normal levels. Their norepinephrine levels decreased substantially too. 

I would think that indicates that Clonidine is a pretty good treatment for it. 

Propanolol didn't do much when the BP and heart rate was low in patients. But when they had tachycardia it would decrease the HR by about 12 beats a minute. 

The study says that most of the patients could be treated with Clonidine and that many of them could eventually decrease the dosage after 2-4 years, and go onto diazepam 5mg three times a day.

It refers to an earlier study that concluded that baroflex failure could be differentiated from pheochromocytoma because pheochromocytoma doesn't respond to clonidine treatment. 

They concluded the paper by saying that baroflex failure symptoms range from patients that have an acute hypertensive crisis to patients that have what they called habitual volatility of blood pressure and with heart rate and high BP surges in response to stress. But they could be punctuated by periods of normal or even low BP during rest. And that it is important to differentiate baroflex failure from other causes in order to treat the orthostatic HYPERtension properly. 

I've done the best I can to condense this study. If you want to read it yourself here is a link to it. New England Journal of Medicine: The Diagnosis and Treatment of Baroreflex Failure

Here is some information about people who are "extreme dippers" This medical journal article says that  these extreme drops in BP at night are closely related to the abnormalities of autonomic nervous activity.

They defined orthostatic HYPERtension slightly differently than other places. They said it was a systolic BP rise of 10 mmHg when in the upright or standing position after 6-10 minutes. Whereas the above definitions required a 20 mmHg raise. 

It also says that orthostatic HYPERtension isn't well known and that it hasn't been well defined. Some places define it as a rise from 90mmHg to above 90 mmHg. And that the older patients had higher systolic BP and lower diastolic BP. 

I would assume this study would be a good one to point out to your doctor if you have a tilt table test and they say it was negative. American Heart Association: Relationship Between Extreme Dippers and Orthostatic Hypertension in Elderly Hypertensive Patients  (72%) of the 14 extreme dippers had orthostatic hypertension, and (27%) of the 11 extreme dippers had orthostatic hypotension. 

This study concluded that: Orthostatic hypertension is a novel complication in normotensive diabetic patients and may associate with early stage neuropathy and development of sustained hypertension. American Diabetes Association: Orthostatic Hypertension in Patients With Type 2 Diabetes

"Although orthostatic hypotension is well recognized and commonly encountered, there are only a few reports of orthostatic hypertension. Most of the reported cases of orthostatic hypertension were related to excessive venous pooling, with an initial drop in cardiac output followed by overcompensation with an excessive release of catecholamines, or to nephroptosis, (also called floating kidney or renal ptosis is an abnormal condition in which the kidney drops down into the pelvis when the patient stands up.) with orthostatic activation of the renin-angiotensin system."

In this study, again from the American Heart Association, the patient had normal plasma and urinary catecholamines and renin release. Pharmacological tests of autonomic nervous system function showed an increased pressor sensitivity to norepinephrine (11 to 14 times normal), normal sensitivity to isoproterenol, diminished baroreceptor reflex sensitivity, and exquisite sensitivity to alpha blockers. This unusual case of orthostatic hypertension appears to be secondary to vascular adrenergic hypersensitivity. It also goes with the rise from 90 mmHg to above 90 mmHg on upright position. And refers to a man who had previously had orthostatic HYPOtension that was followed by HYPERtension. That is precisely what happened to me. 

"Autonomic evaluation demonstrated diminished baroreflex sensitivity and an exaggerated pressor response to the cold pressor test and Valsalva phase 2. Combined autonomic blockade with propranolol and atropine abolished the postural BP changes. These data suggested that the exaggerated pressor response was due to an increase in systemic vascular resistance in excess of the demands arising from a transient postural fall in cardiac output."

They used the Valsalva maneuver to indicate baroflex failure. It is the action of attempting to exhale with the nostrils and mouth, or the glottis, closed. This increases pressure in the middle ear and the chest, as when bracing to lift heavy objects, and is used as a means of equalizing pressure in the ears. When you do that your pulse is supposed to drop when you let your breath out. 

Just out of curiosity, I did this and my heart rate actually went up instead of down after I let my breath back out. I have one of those wristbands that measures your heart rate and steps and how many hours you are sleeping. It made my ears pop a lot. I repeated it and got the same response. (not a picture of me)

 "Orthostatic hypertension is also associated with morning blood pressure surge and extreme nocturnal blood pressure dipping, both of which increase the pulsatile haemodynamic stress of central arterial pressure and blood flow in patients with systemic haemodynamic atherothrombotic syndrome."Orthostatic hypertension—a new haemodynamic cardiovascular risk factor

There is still a lot of information here to absorb, even after I attempted to condense it. But I hope it is helpful to someone. I will be rereading it myself to try and get a grip on it. 

I appologize for some of the weird formatting of this post. I don't know if it is something wrong with blogger or is related to the photos I included or what. I attempted to fix it about six times.

Don't forget to watch the POTS videos at the bottom of the blog. You have to scroll down for them.