I have and still carry the diagnosis of Sjogren's, but what I have and also am diagnosed with is Autoimmune Autonomic Ganglionopathy.

I DO have musculoskeletal pain, but it is in the fascia. I have 2 torn rotator cuffs, bilateral hip bursitis, both knees have been shot up for tendonitis, both feet plantar fasciitis, both wrists and hands, tendonitis and arthritis. Basically my fascia is very inflamed and if I do not drink like a water buffalo, I can not move as these tissues need lubrication. It's 'Sjogren's' in all the fascia.

Yes, eyes and mouth are dry. Also gastroparesis, esophageal issues, severe constipation, odd MS like sensations such as numbness of both legs up to the hips at times. Extreme fatigue or the sensation of being unable to get up without maximal effort, then when standing, I see spots and get tunnel vision. I get 'coat hanger' headaches which are migraine in quality. Muscle spasms, fasciculations, arrhythmias, raynauds like pain when exposed to cold, intractable insomnia, anhidrosis you name it, I got it. Why? Because the only organ that can cause this much misery is your sensory nervous system-autonomic nervous system...it controls it all. PLUS, most medications make matters worse. I have found the less the better for me right now. My body is so sensitive, I can feel when I take a Tylenol. Yes, I do occasionally take something stronger, but it is few and far between.

Autoimmune Autonomic Ganglionopathy is FINALLY starting to get some press. When I got my diagnosis, I couldn't believe there was a Dysautonomia as a stand alone disease. There is and it takes an autonomic specialist to find it because Rheumatologists don't do neurology. My ANA is positive, and I believe that the positive ANA I have is because the test is picking up the neuronal antibody that is attacking my small fibers and dorsal root ganglion.

I don't think I have Sjogren's Syndrome and for a long time I did. Then the doctor said I was way beyond the scope of that. So, I'm OK with the new face of the old symptoms. For me, it's AAG. To help others understand, I tell them it's like Lupus but attacks the autonomic nervous system.

Also, depending on the specialty you see, that is what you get diagnosed with. If you don't have a +ENA, even with a +ANA, rheumatology tends to not want to deal with you. If you see a neuro without a background or interest in autonomic disorders, even with a + ENF biopsy they don't do much for you. I do think that if you can get to an autonomic disorders center, it helps. If you have autonomic testing and it shows abnormality, and you have a + ANA, it's pretty evident that you have an autoimmune neuro condition.

All possible diseases must be ruled out so a treatable cause isn't omitted.

Treatments for AAG, are very similar to treatments for autoimmune disease EXCEPT for drugs that can harm the nervous system, such as methotrexate, azothiaprine etc.

I have to ask...if you have AAG, then what is causing the 'autoimmune' aspect, if not Sjogren's? Do you have another AI disease? I too have AAG, but have been told that Sjogren's is the autoimmune component that has attacked the autonomic and dorsal root ganglia...which in turn causes the dysautonomia and PN.

I do think the definition of Sjogren's will change in time as it's scope goes way beyond what is typically associated with Sjogren's. About time they stopped tagging dry eyes and mouth and started talking about the true damage at the cellular level that affects every organ and connective tissue. Hard to find a doctor that understands what Sjogren's really IS.

It's been a long time since we discussed this...good to have you back!!

Autoimmune conditions span the gamut. AAG IS the underlying disease.

It took me a long time to get that thru my head.

AAG is a stand alone autoimmune disease.

The elevated ANA is caused by a yet unidentified antibody, hence a negative ENA.

Dry eyes and dry mouth are the product of a malfunctioning nerve to those organs and it will show up as lymphocytic infiltrate in a labial biopsy....because the nerves are undergoing the same inflammation as is present in connective tissue disease.

I have read that AAG is the sensory version of ALS (which is motor.) ALS affects the anterior horn, AAG the dorsal root. One is motor, one is sensory. Totally different diseases, but similar in that they are a degeneration of the nerve roots at the spine. ALS isn't secondary to any other disease, it just is. Same with AAG. It just is.

I'd love to see what you've read on this. Do you have any links you can share??

That would actually make some sense for me (AAG as a stand alone) since my SSA & SSB are negative, yet my lip biopsy was positive.

They have confirmed the dorsal root damage in my case with a new MRI/MRN at Hopkins that looked at the DRG itself. It showed bilateral enlargement along with increased signal consistent with ganglionitis.

But my neuro (who is also a rheumy---who's practice is devoted to those with neuro complication of rheumatic disease) says that the AAG is the "result" of the Sjogren's attack on the autonomic ganglia and DRG. But I can see 'some' of your logic. However, the lip biopsy looks at the minor salivary glands, not nerves...so the infiltrates present are within the salivary glands itself. Of course neuropathy can cause simple dry mouth & eye (from nerve damage), but you'd have to have an autoimmune process attack the glands itself for infiltrates to show in lip biopsy. That would be Sjogren's, not AAG...unless AAG is known to attack salivary glands.

I am seeing him (neuro) on the 27th as I now have what appears to be psoriatic arthritis. He is open to discussion so I will present this 'stand alone' AAG to him. Would love to read and take some documentation with me, if you can forward some links.

Very interesting, indeed.

Depends what specialty you see. I was told the infiltrates of my biopsy did NOT affect the gland. I have seen at least 10 rheumatologists since 2000 when my biopsy was done and most of them blew off my biopsy. Some looked at it a bit more closely after my ANA went positive.

AAG is definitely a stand alone disease.

Autoimmunity can attack any organ system, but when it attacks the autonomic system, it attacks the control center of the entire body, hence causing a huge amount of symptomotolgy.

Rheumatology has yet to define many of it's diseases very well. Sjogren's is everything from sicca to AAG.

This is very interesting information for me, as I have such a myraid of symptoms with a strong family history of autoimmune disease. I have a negative ANA but positive acetylcholine ganglionic receptor antibodies. My ANA was low positive 15 yrs ago, and with mildly dry eyes & mouth with sensory neuropathy just beginning, they thought maybe Sjogren's. I took Plaquenil for about 9 yrs and ANA was always negative after that. SSA/SSB always negative. I stopped taking Plaquenil about 3 yrs ago since my nerves kept getting worse but nothing showing in my bloodwork.

Is there a way to find a neurologist-rheumatologist-autonomic specialist without going to the Mayo or Hopkins?! My doctors have no clue & don't seem to care much. I like my neuro as a person, but when I showed him the Liza Jane spreadsheet and suggested we may be missing something, he just smiled & said I've had a thorough workup. I feel like I'm wasting time, money & sanity.

My 17 yo was just diagnosed with mild POTS via a tilt-table test, I know I have this without having to be tested.

My advice is to search out an autonomic specialist for diagnosis and treatment....I have gone that route and right now, I am in a holding pattern until I get a new neuro...my old neuro left the area. Your profile sounds to me, like AAG. I was on Plaquenil and broke out in raised red bumps, and it didn't help anyway. I haven't found anything that is tremendously helpful other than doses of prednisone that can't be sustained. :/ (I have not yet done a TNF blocker or plasmaphersis and right now I am stable enough not to mess with myself) That is not to say I have a normal quality of life, but, I am able to move around, sleep, eat, drink and be merry on occasion. I am managing symptoms and trying very hard not to take much for meds because what is supposed to help one thing always screws up something else.

To Billylyne5
Thanks for starting this informative thread - I don't have much spare time (or brain energy) to search for answers to medical 'mysteries' so it's great when I come across a thread with lots of info. Hope you're making progress with your quest for a diagnosis. Hang in there.
To all who have posted thanks for the info, especially about AAG - even if some of it is beyond my level of medical-speak comprehension it is very useful and I will research further.

Specifically to Synnove re the "tightness in the chest" this sounds similar to a symptom (amongst many) I have been experiencing for a number of years. Some questions: Is it in the upper region of the chest - sort of lower oesophageal area? Does it come and go with no apparent reason or cause? Does the duration vary from hours to sometimes days or even weeks? Does the tightness at times feel like a burning choking that sometimes leaves you short of breath if you over exert yourself? Sorry for all the questions but it's a difficult symptom to describe. And also sorry for not being able to definitively attach the symptom to a specific diagnosis.
In my case I suspect that I may also have Sjogrens (as a secondary auto-immune condition to primary Addison's Disease (adrenal failure) - diagnosed 5 years ago this week). My father also had Sjogrens (secondary to RA) so I am familiar with some of the presenting symptoms. Unfortunately I'm in a country with a Public Health system so getting to see a specialist is difficult and if a test shows up negative - that's it! they stop looking! Have to keep pushing and looking for answers - won't go into all my other symptoms here but also suspecting some type of Peripheral Neuropathy. Had a confirmed bout of Trigeminal Neuralgia last year.
Also in regard to your other symptom "waking up at night with a rush like symptom that moves out over different body parts, accompanied by a sensation of warmth?" This sounds like it may be something called L'Hermite's Sign. It's a nerve spasm (bit like an electric shock) - often originating in the spine - it can be strong enough to wake someone from a deep sleep but goes quickly and doesn't normally leave any trace. If there are other symptoms with it eg weakness, or stroke signs definitely get it checked.
Hope this is of use to you and others.
at the same time

bluesfan,

I am sorry to hear you have Adison's. I hope you get referral to both neurology and rheumatology doctors to check out possible neuropathy and underlying causes, and to explore your suspicions of a Sjoegren's diagnosis.
I understand your dilemma re. healthcare systems, I lived in 2 countries with socialized medicine.

Regarding your question on the chest symptoms: My symptoms is described as a tightness,squeezing and heavy feeling. Symptoms are both anterior (front) and posterior (back). It is right across the chest, usually at mid point and up in upper chest area, and leaves me with feeling of heaviness in chest and tightness across upper back. Not really short of breath, but somehow having the feeling to expand the chest. Sometimes it comes from no apparent reason, other times not. Symptoms come even without physical strain. Sometimes it comes in the afternoon when the sensory neuropathy symptoms get worse. It last for a few hours. I get it 3 -4 times out of 7 days.
There is no burning like you described. ( I have other type of burning at same time, but that is something else.)
The upper chest you mentioned as burning could be acid reflux. I know doctors seem to often brush a lot of symptoms away saying " oh, that is just acid reflux"
I would recommend you also get a GI, gastrointestinal specialist.

BUT, acid reflux can play a part in the big "symphony" of neuropathy. If you have SFN, most likely there is also autonomic neuropathy involved. This can again cause all sorts of GI problems like extreme acid reflux, gastroperesis.
This also happened to me.
I needed a special GI to do a test called Mannometry ( could be mis spelled)


Regarding the suspicion of Sjoegren"s that both you and I have described in our posts: I do not know, it is my thinking that perhaps the chest tightness can also have something to do with lack of secretion for lubrication in lungs, perhaps caused by Sjoegren"s???
I know, that for me, sometimes a hot steamy shower helpes.
I also have a cardiac issue that may be playing a role in this. As en bloc described. I will discuss my autoimmune disorder with the cardiologist soon.

en bloc has desribed in his posts how many systems can get attacked from autoimmune disorders, Sjoegre"s being one of them, like the cardiovascular system, respiratory system, nervous system and others, for example the cutaneus system (skin)

You mentioned you thought you had peripheral neuropathy. I have over the time coping with my different symptoms and diagnosis that with Sjoegren"s one of the typical symptoms is severe neuropathy.
--Do you have pins and needles, burning sensation, numbness typical for SFN?
-- I also have the tingling up and down a nerve path and I know this is not SFN but from a long nerve. I have SFN in addition.

Billylyne5,
let us know when you have had your appointment with the rheumatologist!
Thanks for reading