Hi there - Thanks for your message. I have had an extensive work up for Sjogren's. I first was diagnosed with it by a doc at Harvard Medical Center in 2002 based on signs and symptoms. I had failed the Schirmer's test, but all other tests were normal. Just this past summer, I went to the Sjogren's Syndrome Center at National Institutes of Health in Bethesda, MD. They did a lip biopsy, which showed inflammation, but not the exact markers for sjogren's as I understand it. They also did a saliva test (both provoked and unprovoked). I was able to make saliva when provoked. I also had an eye exam. I produced plenty of tears on the Schirmer's test so I didn't meet the eye criteria.

They have a strict criteria (I think it is called the American European Protocol??) for diagnosing Sjogren's and I did not meet the criteria. You are right that you can be seronegative and still meet the criteria. I am seronegative, but I also failed to meet the other criteria so they said that, for now, they cannot diagnose me with Sjogren's. They said that doesn't mean I won't meet the criteria later. I just don't now. They said there is a correlation between Sjogren's and Autonomic Nervous System dysfunction, but they just don't understand it completely yet.

I have not yet had a skin biopsy/muscle biopsy for the neuropathy, but I plan to pursue that next. I have found a neurologist willing to do it, but he wants to do some MRI scans first. I think he should just go straight to the skin biopsy. I don't think the MRIs are going to show anything. They didn't 13 years ago when I first had the neuro work up.

I'm curious. What type of doctor did your skin biopsy? Was it a surgeon or another specialty? As I understand it, they do a punch biopsy, but how deep is it? I want someone who is experienced in this to do it.

Also, I have read about some tests they do for autonomic nervous system, but I don't know all of them. Can you enlighten me on this? I don't know my way around this site very well yet so any help you can give is appreciated.

Thank you again for replying to my post.

--to determine intraepidermal small-fiber nerve density and condition is a very quick and non-invasive procedure--small amounts of local anesthetic are injected at the sites and then a small circle of skin, about 3mm in diameter and 1 mm in thickness, are excised. The whole procedure takes only a few minutes; samples are usually taken from the calf near the ankle, from the proximal thigh, and often from the arm near the elbow, to check if there is a distal to proximal gradient or "die back" (nerves in lower density or worse condition farther away from the center of the body). A few band-aids and one is done.

These samples are then sent to specialty labs at which special stains are used to bring out the nerves and then they are examined by electron microscopy. Not many places do this--Johns Hopkins, Jack Miller in Chicago, Cornell-Weill/Columbia Presbyterian, Massachusetts General are among the more well-known that have the facilities, and the trained pathologists, to do so.

As for the Sjogren's. If you don't have positive blood work, then a positive biopsy is a must. There are set guidelines for the grading of lip biopsies. The Greenburg Scale is the one used by Hopkins and I also believe NIH. You can have inflammation present, but in order to meet the criteria, you must have at least one focus (focus = an aggregate of 50 or more lymphocytes with 4 mm sq). Therefore, you likely had infiltration of lymphocytes, but not a complete focus. But like you said, that change change later.

The neurologist at Hopkins did my skin biopsy, but I understand dermatologist have been trained to do them as well. However, they must be sent to one of the few places capable of reading them...like Glenntaj said.


Autonomic testing can include:

gastric empty study for delayed stomach emptying (eating some scrambled eggs with contrast and laying still while pictures tracking movement are taken for 2 hours)

Tilt table for diagnosis of POTS (postural orthostatic tachycardia syndrome) and NMH (neurally mediated hypotension).

cardiac holter monitor and/or event recorder to confirm heart rate/rhythm dysfunction

QSART (sweat test)

You have a vast array of symptoms which could be autoimmune. I would go ahead with the MRI"s just to be sure to rule out structural problems that may not have been present years ago. There is a new MRI to look at the dorsal root ganglia. Ganglionitis has been connected to Sjogren's. However, this new protocol is likely not available in many places...mine was done at Hopkins.

Diagnosis can take a long time. Sounds like they have looking for quite some time already. Hopefully the MRI's or skin biopsy will hold answers for you.

Thank you for the info, especially on the autonomic nervous system testing. The only tests I've had of these so far is the gastric empty studies. I've had two and both have come back normal. The most recent one was a few months ago.

Thanks again.

the neg test for Celiac is not the end of that, i am afraid. There is ~ 20% false negative n the blood work, and that is if it has been completely done!
And it could be that you hadn't progressed far enough in the disease process that you didn't test positive. The only real "test" for celiac/gluten sensitivity is the an absolute gluten free diet for a fairly extended period of time, 6 months or so for neuro symptoms. Good luck

Axseptants,

Although not glad your EMG showed problems, at least you have a few answers and a direction to head now.

Thank you so much. Also, today, I got some interesting news. I received results of an EMG in my legs I had in March. I haven't talked to the doctor yet, but I picked up the results.

Here's the narrative:
Summary: The nerve conduction study of the above nerves showed prolonged DML right peroneal nerve, decreased amplitude right peroneal at the fibular head, decreased conduction velocity right tibial. The monopolar needle EMG revealed increased in spontaneous activity and positive wave vastus lateralis.

Impression: This is an abnormal study. There is electromyographic evidence of sensory and motor polyneuropathy and possible myopathy. Etiology is yet to be determined. Clinical correlation is required.

I had given up on neurologists over a decade ago when all my test results came back normal. I just started going down the neuro route again recently. 16 years since my symptoms started and I finally get an EMG that shows something. I guess I should still have the skin biopsy too to determine the small fiber nerve aspect of the damage??

Thank you again.