Since we discovered that my POTS is caused by an autoimmune disease (Sjogren's Syndrome) that is attacking my autonomic nerves, sensory nerves, motor nerves and other important parts of me, I started researching the connections between dysautonomia and autoimmune diseases. I want to share some of this information with my fellow POTSies. Out of the 500,000 to 1 million of us out there, I am sure there are other POTS patients who have an undiagnosed autoimmune disease. I know I'm not the only one who has POTS is caused by an autoimmune disease.
I saw numerous so called "POTS experts" who told me there was no way to identify the cause of my POTS and that I shouldn't bother looking because it was just "idiopathic" - which means of unknown origin. They also said it would "probably just go away on its own in 5-10 years." I wasn't planning to sit back and watch my life go by from the couch for the next 10 years. I made it my mission to figure out the cause of my POTS. For starters, I knew that my POTS was caused by autonomic neuropathy. So what I really wanted to know is what had caused my nerves to die off, and I also wanted to know whether it was a one time nerve injuring event, or whether the damage was ongoing or progressive. I think these are very rational and reasonable things to ask, especially if your nerves have been damaged to the point where you can barely walk for over a year. For asking these simple two simple questions (what caused it and is it progressive), I was scoffed at, ignored, dismissed and given the old fashioned eyeball roll by more than a handful of doctors claiming to have expertise in POTS (neurologists and cardiologists). How dare I want to understand what is going on in my body! How dare I question their lazy diagnosis of "it's idiopathic."
While I understand that some forms of neuropathy are idiopathic because modern medicine just hasn't figured out all of the causes of neuropathy yet, I refused to accept an "idiopathic" neuropathy diagnosis until my doctors at least checked me out for all of the known causes of neuropathy. I didn't think it was fair to label it "of an unknown origin" until we had ruled out all of the known causes of neuropathy. Sure enough, when I finally found a doctor who was willing to test me for the known causes of neuropathy, he figured me out in a few hours. My POTS is caused by an underlying autoimmune disease, Sjogren's Syndrome, that is attacking my autonomic nerves, as well as my sensory and motor nerves, and some of my major organs (lungs, heart, kidneys, stomach, etc.). And Sjogren's is progressive - it can even lead to lymphoma. So if I had just listened to those so called POTS experts who told me not to bother looking for the cause of my POTS and just to go home and take some beta blockers, I would have continued to get sicker and sicker.
While I feel a sense of outrage that it was so hard to get a doctor willing to help figure me out, it also saddens me, because I know there are probably hundreds, if not thousands of other POTS patients who have been told their POTS is idiopathic and "will just go away on it's own," when their POTS may actually because by a serious and progressive autoimmune disease.
If your POTS is caused by an underlying autoimmune disease, there are specific medications you can take to stop and maybe even reverse your autoimmune problems, which would prevent your POTS from getting worse over time, and could even help you fully recover.
The most well known autoimmune disease that causes POTS and similar dysautonomic symptoms is Guillian Barre. Guillan Barre is a something like an infection and autoimmune disease in one, usually with an acute onset, that results in inflammation of the nerves and damage to the protective myelin sheath that covers the nerves. This results in the nerve signals traveling too slow or a total loss of the nerve fiber. It commonly occurs after lung or GI infection, but the infection may be gone by the time the nerve damage occurs. There is almost always some loss of motor function, due to damage to the motor nerves.
A close cousin of Guillan Barre is CIDP, Chronic Inflammatory Demyelinating Polyneuropathy. CIDP is consider to be the chronic form of Guillian Barre. My neurologist at Cornell Presbyterian in NYC, Dr. Norman Latov, happens to be one of the world's leading experts on CIDP.
Another autoimmune disease, the one I have - Sjogren's Syndrome, can cause autonomic, sensory, and motor neuropathy, and CNS lesions/inflammation in the spinal cord and brain. And Sjogren's doesn't just attack nerves - it can attack your lungs, kidneys, bladder, joints, oral and eye mucosa, GI tract, etc. I have all of the classic PD POTS (partial dysautonomic POTS) symptoms - hypotension, tachycardia, low blood volume, GI problems, trouble swallowing, headaches, achy neck/shoulders/back, shooting pain in my arms and legs, oversensitivity to light/noise/skin sensations, etc. - and all of those symptoms can be caused by the nerve damage caused by Sjogren's.
Various forms of Lupus can cause dysautonomia, as explained here.
Mutiple Sclerosis (MS) can sometimes cause dysautonomia, as discussed here.
Ehlers Danlos Syndrome (EDS), while it isn't really an autoimmune disease, is considered a connective tissue disease - just like Lupus and Sjogren's. Many POTS patients have EDS and many EDS patients have autonomic neuropathy.
Celiac Disease is associated with dysautonomia and neuropathy, as discussed in this article. Millions of Americans have small fiber neuropathy (pins, needles, numbness or burning in the feet), and about 16% of people with small fiber neuropathy have Celiac Disease, although many of them don't know it.
Crohn's Disease, Addison's Disease, Graves Disease, Hashimoto's Thyroiditis and other autoimmune disease have been associated with dysautonomia.
Many people with one autoimmune disease end up having more than one autoimmune disease as time goes on, so it is not uncommon for someone with Sjogren's to also have Lupus, or someone with Crohn's to also have rheumatoid arthritis.
Some forms of diabetes are also considered to be autoimmune in origin. And diabetes is the most common cause of neuropathy. You may here the phrase "diabetic neuropathy" and that is used to refer to the sensory and sometimes autonomic neuropathy experienced by people with diabetes. Careful control of blood sugar levels and overall health can prevent and sometimes reverse neuropathy in diabetic patients.
Small fiber nerves, which are the farthest points on the peripheral nerve system, can grow back at 1 millimeter per year under ideal conditions. However, if an autoimmune disease is continuing to attack the nerves, it is unlikely they will be able to regenerate. You would have to get the autoimmune process that is attacking the nerves to stop - and that can be accomplished with medications in many cases.
There are many antibodies that are known to damage autonomic nervous system. Here is a short list. There are MANY more and scientists are discovering new antibodies all the time:
Alpha-fodrin
ANA
ENA
ANNA1 (also called anti-Hu)
ANNA2
ANNA3
Anti-Muscarinic Receptor
Anti-Neurolnal
Anti-Smith
Anti-Ro (also called SS-A)
Anti-La (also called SS-B)
RF (rheumatoid factor)
Ganglionic Acetylcholine Receptor Autoantibody (AChR)
CRMP-5 IgG
GAD-65
Striational antibodies
Anti-calcium channel antibody
Acetylcholine receptor muscle binding antibody
Acetylcholine receptor ganglionic neuronal antibody
Acetylcholine receptor modulating antibody
Neuronal voltage-gated potassium channel antibody
PQ-type calcium channel antibody
Amphiphysin antibody
There is an interesting presentation on the Mayo Clinic website from February 2011 pertaining to Mayo's paraneoplastic antibody panel. Any neurologist who is worth his/her salt with a dysatuonomic patient will send a few vials of that patient's blood up to Mayo to have this panel run. Your local lab cannot do it. Mayo is the only place that can do it, but your local lab can send your blood to Mayo's lab.
Paraneoplastic syndromes are when a tumor, usually small cell lung cancer, causes antibodies to develop that cause damage to your nerves. Sometimes the tumor is so small, the tumor itself isn't causing any symptoms or isn't even large enough to be seen on a scan. Sometimes the neurological symptoms developed and are identified by a doctor before the cancer. That is why it is important to check all neuropathy patients for this.
Interestingly, many POTS patients like myself can point to a specific date or week during which they became terribly sick. I woke up sick January 2, 2010 while on a ski trip. This could have been a sudden onset viral infection, and such infections are among the known cause of POTS. However, severe viral infections are also known to trigger or "turn on" autoimmune diseases that a person may have been predisposed to due to genetic factors. My doctors think that I probably caught a virus, which is why I had such a sudden onset illness, and that the virus triggered a massive immune system response in my body, and then that immune system imbalance morphed into Sjogren's Syndrome.
The good news is that, if your dysautonomia is caused by or associated with an autoimmune disease, there are lots of drug therapies that target the underlying immune problem cause of the dysautonomia, rather than just treating the dysautonomia symptoms like most doctors do with beta blockers, Flourinef, Midodrine, etc. My next post will be about those autoimmune therapies, especially the great success seen when "idiopathic" dysautonomia, mostly like caused by an unidentified infectious and/or autoimmune problem, is treated with Intravenous Immunoglobulin (IVIG).
I saw numerous so called "POTS experts" who told me there was no way to identify the cause of my POTS and that I shouldn't bother looking because it was just "idiopathic" - which means of unknown origin. They also said it would "probably just go away on its own in 5-10 years." I wasn't planning to sit back and watch my life go by from the couch for the next 10 years. I made it my mission to figure out the cause of my POTS. For starters, I knew that my POTS was caused by autonomic neuropathy. So what I really wanted to know is what had caused my nerves to die off, and I also wanted to know whether it was a one time nerve injuring event, or whether the damage was ongoing or progressive. I think these are very rational and reasonable things to ask, especially if your nerves have been damaged to the point where you can barely walk for over a year. For asking these simple two simple questions (what caused it and is it progressive), I was scoffed at, ignored, dismissed and given the old fashioned eyeball roll by more than a handful of doctors claiming to have expertise in POTS (neurologists and cardiologists). How dare I want to understand what is going on in my body! How dare I question their lazy diagnosis of "it's idiopathic."
While I understand that some forms of neuropathy are idiopathic because modern medicine just hasn't figured out all of the causes of neuropathy yet, I refused to accept an "idiopathic" neuropathy diagnosis until my doctors at least checked me out for all of the known causes of neuropathy. I didn't think it was fair to label it "of an unknown origin" until we had ruled out all of the known causes of neuropathy. Sure enough, when I finally found a doctor who was willing to test me for the known causes of neuropathy, he figured me out in a few hours. My POTS is caused by an underlying autoimmune disease, Sjogren's Syndrome, that is attacking my autonomic nerves, as well as my sensory and motor nerves, and some of my major organs (lungs, heart, kidneys, stomach, etc.). And Sjogren's is progressive - it can even lead to lymphoma. So if I had just listened to those so called POTS experts who told me not to bother looking for the cause of my POTS and just to go home and take some beta blockers, I would have continued to get sicker and sicker.
While I feel a sense of outrage that it was so hard to get a doctor willing to help figure me out, it also saddens me, because I know there are probably hundreds, if not thousands of other POTS patients who have been told their POTS is idiopathic and "will just go away on it's own," when their POTS may actually because by a serious and progressive autoimmune disease.
If your POTS is caused by an underlying autoimmune disease, there are specific medications you can take to stop and maybe even reverse your autoimmune problems, which would prevent your POTS from getting worse over time, and could even help you fully recover.
The most well known autoimmune disease that causes POTS and similar dysautonomic symptoms is Guillian Barre. Guillan Barre is a something like an infection and autoimmune disease in one, usually with an acute onset, that results in inflammation of the nerves and damage to the protective myelin sheath that covers the nerves. This results in the nerve signals traveling too slow or a total loss of the nerve fiber. It commonly occurs after lung or GI infection, but the infection may be gone by the time the nerve damage occurs. There is almost always some loss of motor function, due to damage to the motor nerves.
A close cousin of Guillan Barre is CIDP, Chronic Inflammatory Demyelinating Polyneuropathy. CIDP is consider to be the chronic form of Guillian Barre. My neurologist at Cornell Presbyterian in NYC, Dr. Norman Latov, happens to be one of the world's leading experts on CIDP.
Another autoimmune disease, the one I have - Sjogren's Syndrome, can cause autonomic, sensory, and motor neuropathy, and CNS lesions/inflammation in the spinal cord and brain. And Sjogren's doesn't just attack nerves - it can attack your lungs, kidneys, bladder, joints, oral and eye mucosa, GI tract, etc. I have all of the classic PD POTS (partial dysautonomic POTS) symptoms - hypotension, tachycardia, low blood volume, GI problems, trouble swallowing, headaches, achy neck/shoulders/back, shooting pain in my arms and legs, oversensitivity to light/noise/skin sensations, etc. - and all of those symptoms can be caused by the nerve damage caused by Sjogren's.
Various forms of Lupus can cause dysautonomia, as explained here.
Mutiple Sclerosis (MS) can sometimes cause dysautonomia, as discussed here.
Ehlers Danlos Syndrome (EDS), while it isn't really an autoimmune disease, is considered a connective tissue disease - just like Lupus and Sjogren's. Many POTS patients have EDS and many EDS patients have autonomic neuropathy.
Celiac Disease is associated with dysautonomia and neuropathy, as discussed in this article. Millions of Americans have small fiber neuropathy (pins, needles, numbness or burning in the feet), and about 16% of people with small fiber neuropathy have Celiac Disease, although many of them don't know it.
Crohn's Disease, Addison's Disease, Graves Disease, Hashimoto's Thyroiditis and other autoimmune disease have been associated with dysautonomia.
Many people with one autoimmune disease end up having more than one autoimmune disease as time goes on, so it is not uncommon for someone with Sjogren's to also have Lupus, or someone with Crohn's to also have rheumatoid arthritis.
Some forms of diabetes are also considered to be autoimmune in origin. And diabetes is the most common cause of neuropathy. You may here the phrase "diabetic neuropathy" and that is used to refer to the sensory and sometimes autonomic neuropathy experienced by people with diabetes. Careful control of blood sugar levels and overall health can prevent and sometimes reverse neuropathy in diabetic patients.
Small fiber nerves, which are the farthest points on the peripheral nerve system, can grow back at 1 millimeter per year under ideal conditions. However, if an autoimmune disease is continuing to attack the nerves, it is unlikely they will be able to regenerate. You would have to get the autoimmune process that is attacking the nerves to stop - and that can be accomplished with medications in many cases.
There are many antibodies that are known to damage autonomic nervous system. Here is a short list. There are MANY more and scientists are discovering new antibodies all the time:
Alpha-fodrin
ANA
ENA
ANNA1 (also called anti-Hu)
ANNA2
ANNA3
Anti-Muscarinic Receptor
Anti-Neurolnal
Anti-Smith
Anti-Ro (also called SS-A)
Anti-La (also called SS-B)
RF (rheumatoid factor)
Ganglionic Acetylcholine Receptor Autoantibody (AChR)
CRMP-5 IgG
GAD-65
Striational antibodies
Anti-calcium channel antibody
Acetylcholine receptor muscle binding antibody
Acetylcholine receptor ganglionic neuronal antibody
Acetylcholine receptor modulating antibody
Neuronal voltage-gated potassium channel antibody
PQ-type calcium channel antibody
Amphiphysin antibody
There is an interesting presentation on the Mayo Clinic website from February 2011 pertaining to Mayo's paraneoplastic antibody panel. Any neurologist who is worth his/her salt with a dysatuonomic patient will send a few vials of that patient's blood up to Mayo to have this panel run. Your local lab cannot do it. Mayo is the only place that can do it, but your local lab can send your blood to Mayo's lab.
Paraneoplastic syndromes are when a tumor, usually small cell lung cancer, causes antibodies to develop that cause damage to your nerves. Sometimes the tumor is so small, the tumor itself isn't causing any symptoms or isn't even large enough to be seen on a scan. Sometimes the neurological symptoms developed and are identified by a doctor before the cancer. That is why it is important to check all neuropathy patients for this.
Interestingly, many POTS patients like myself can point to a specific date or week during which they became terribly sick. I woke up sick January 2, 2010 while on a ski trip. This could have been a sudden onset viral infection, and such infections are among the known cause of POTS. However, severe viral infections are also known to trigger or "turn on" autoimmune diseases that a person may have been predisposed to due to genetic factors. My doctors think that I probably caught a virus, which is why I had such a sudden onset illness, and that the virus triggered a massive immune system response in my body, and then that immune system imbalance morphed into Sjogren's Syndrome.
The good news is that, if your dysautonomia is caused by or associated with an autoimmune disease, there are lots of drug therapies that target the underlying immune problem cause of the dysautonomia, rather than just treating the dysautonomia symptoms like most doctors do with beta blockers, Flourinef, Midodrine, etc. My next post will be about those autoimmune therapies, especially the great success seen when "idiopathic" dysautonomia, mostly like caused by an unidentified infectious and/or autoimmune problem, is treated with Intravenous Immunoglobulin (IVIG).