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.

Both Billylyne5 & Jenng,

Finding an autonomic specialist isn't easy...there just isn't a lot of good ones out there. I waited 9 months for an appointment to my new neuro that is autonomic specialist. They are just few and far between.

I have been to Mayo (has probably the best autonomic testing center in the country) and that's where I was diagnosed with my autonomic neuropathy/dysautonomia. But I wasn't impressed with their neurologist dept doctors at all!!

Start looking at an academic hospital. It would be best if you could get a 'team' with neuro (possibly autonomic specialist) and rheumatologist in same system.

If your insurance would cover it, consider Hopkins to the Sjogren's Center and seek out Dr. Birnbaum (he's both a neuro and rheumy---only one in the country) who specializes in treating patients with neuro manifestations of rheumatic disease. You can set up appointment, get evaluation and he will work with your local doctors for continued care options...where your doctors get his treatment advise for you. This way you only travel the one time and then your doctors know how to follow up with your care. Just a thought.

Billylyne5, you may not have the autonomic symptoms, but might still benefit from the neruo/rheumy team at an academic hospital (or the Sjogen's Center for eval) that can work together to find the underlining cause of your symptoms.

Billylyne5, you may not have the autonomic symptoms, but might still benefit from the neruo/rheumy team at an academic hospital (or the Sjogen's Center for eval) that can work together to find the underlining cause of your symptoms.[/QUOTE]

I am in the UK so our system of referrals is sometimes a bit challenging, however I have found that there is a rheumatologist that is involved in a new research program on sjogrens based at a hospital I can get refered too, unfortunately the neurologist there dismissed my symptoms as functional after a 5 minute appointment, my GP and current neuro both felt this was extremely unlikely and discounted his opinion, so it does mean joined up work between neuro and rhemy may be more difficult.
The first challenge is getting the referral to the rheumatologist.

A tilt table test can be performed in a cardiology lab, that is where my first one was done. Other tests like the QSART and thermoregulatory sweat test require special labs. The other tests I had done at an academic center with an autonomic lab. It's been over 10 years and I would like to have them redone, but, the doctor that did the autonomic test on me left for Rush Presby in Chicago. I'm on an HMO, we just switched insurance and we will see what the new neuro says.


I was seen at Wisconsin Medical College in Milwaukee. I know Mayo has a center. I am sure Chicago has something. Vanderbilt in Nashville is very good and the top guy is Dr. Vernino in Texas, in San Antonio I think.

With insurance the way it is, it is hard to get to an autonomic lab, but you could ask for a tilt table test and a epidermal nerve fiber biopsy and if those are both abnormal, that is substantial confirmation and I would think that they would refer you to an autonomic lab. I haven't had the best continuity of care even tho I was with one neuro for 7 years. A lot less was known about this 10 years ago when I developed it.

AAG is considered quite rare. Part of that IMHO is that it isn't recognized but it is still considered rare. I think they look at if you flunk the tests and if you have neuronal antibodies or a positive ANA with negative ENA and a positive skin biopsy for small fiber neuropathy.

Seen GP blood results still showing elevated liver enzymes although lower than last time. Plus my white count was a bit low again.
After discussing the increase in my symptoms my GP immediately said it sounds much more like it's autoimmune and did an urgent referral to the rheumatologist dept so I have an appointment on the 23rd Jan. My GP did look at sjogrens but she asked me what I knew about SLE and talked about that rather than sjogrens 😳 so another consultant another hospital and no doubt a shed load more tests, such fun.

Billylyne5
I am glad you got ref feral to rheumatologist. I hope you will get help with diagnosing .
I do not want to " steal" you thread, but my comments and questions are in relations to your topic.

en bloc,
I am interested in your input with recommendations of Dr. Birnbaum at Hopkins.
I wonder if I should pursue getting a ref feral up there.
You said this doctor specializes in pt with neuro manifestation of rheumatic diseases? Well, this is just what my local rheumatologist diagnosed me with. After almost 2 years of neuro symptoms, my dx was, rheumatoid arthritis with presenting symptoms as sensory neuropathy and Small Fiber Neuropathy.

I have been tested for Sjoegren 2 - 3 times. Negative SSA and SSB 3 times, negative lip biopsy 2 years ago.
Negative skin bx for SFN, but this is about to be repeated. Had QSART at Mayo, positive for SFN by sweat test, and in addition the severe symptoms.
Sero negative inflammatory arthritis for a while. Was considered for Rituxan treatment, had the VectraDA test for rheumatoid arthritis which came back positive in the mild section, so for now being treated with Leflunomide.

I have now lately had such a flare up, I had to seek up help from my rheumy who gave me a weeks treatment with Steroid.
I had joint pains, and ridiculous, increased Burning pain. I have had such dry eyes the last few weeks, I can not open my eyes in am, I have artificial tears on the night table, to use. I have also had this tightness in chest for over 2 years now. It feels like a tight squeeze that sometimes last for hours, and it often comes at the times when my neuropathy symptoms flares up, I have had all kinds of check ups, as CT ( only a couple of stable nodules in chest) PFT showed mild small airway obstruction. ( but this can be due to many things) Cardiologist has put me on small dose of Norvasc, which should help. I do have some cardiac issues, and I will hopefully have a repeat cardiac Ecco next apt in March. But they really have not come up with the reason of my chest tightness.

Is it possible that it can be due to Sjoegrens?

I am now wondering: with the increased/flareup of veuropathy burning symptoms, with the increased dryness of eyes, the joint pain and the chest tightness, should it be time to reassess and test for Sjoegren's again???

I have a apt. with my ophthalmologist at the end of this month, I wonder if I should ask for a Schirmer test?

Cyclelops,
I have never had a MRN. (I have had many MRI of brain and spine due to numerous problems, both spinal, but mostly cerebral aneurysm) But they have never done MRN to possibly study the cause of my neuropathy. I did go to Mayo. That is almost 2 years ago. They did tilt table, was negative then. They also included the sweat test which was positive. I had Gastric Emptying study which was positive for gastroperesis. Otherwise Mayo did not help me much.

I wonder how I should get the MRN done. I wonder if I should have my rheumatologist refer to this Dr. Birnbaum at Hopkins that en bloc recommended, and perhaps this could be done?

Thanks for reading

Synnove,

If you have tested positive for RA, then Sjogren's be considered 'secondary'. It doesn't always appear on labs, but you can still have the condition. There is a 'process' to get an appointment. The information is below:

http://www.hopkinssjogrens.org/

Read the bio on Dr. Birnbaum at the "Meet our Team" link on the right.

You should definitely asked for the Schirmer's test to be done. It can gauge the level of dryness/tear production in your eyes. It's a bit uncomfortable for a few minutes, but not that bad.