[cyclelops]

I had a labial salivary gland biopsy done and read a while back. It was read as having 'periductal inflammation consistent with Sjogren's'. It was read by a large well known midwest referral institution. It was then later read by an other pathologist and rheumatologist in a nearby state, who said it was not typical of Sjogren's. I was told by letter, from these people that there was indeed something 'wrong' but it did not stem from that abnormal biopsy read as Sjogren's, and I would not get any treatment for autoimmune disease.

Subsequently, my new neuro, went with a diagnosis of autoimmune autonomic neuropathy (with potential seronegative sjogrens) based on European Criteria for Sjogrens, IVIG for 6 months and 16 weeks of IV methlypred via PICC...(omg--do not recommend---I was his only patient that lost 20# on it). It did not affect the course of my disease or my measurements on autonomic tests, as far as improvement goes...now maybe, it made it progress slower, but that is hard to say.

I now read that they are finding amyloidosis on labial gland biopsy and sicca syndrome has some association with amyloidosis. Hmm. I have continued to decline, and when I went and reread my echocardiogram results saw that not just my mitral was regurging, but now also my tricuspid. While they said the mitral was at 2+ with a substantial central jet, the tricuspid is still mild. I still have an acceptable ejection fraction at 55%, with no ventricular hypertrophy as of 3 years ago. MY SOB and presyncope is due to the hypotension that comes with the AN.

Next is a fat aspiration and a skin biopsy of some suspicious lesions in the near future ....and now my neuro wants to see those labial gland slides, which are now over 7 years old, if I can locate them.

You think they would have stained them or examined them for amyloid back then when they did them. Now I hope they still have them. Now I was not diagnosed with PN when I had those done, but that was due to the doctors I had back then just not doing the right tests....I can tell you I had umpteen tests for carpal tunnel in the years they cared for me...I finally got new doctors when I started to have autonomic troubles, but even then it took an additional 3 years to get the small fiber diagnosis and the subsequent autonomic and sensory neuropathy diagnosis.

Have any PNers had salivary gland lip biopsies and run into this situation? I am wondering if they can still identify amyloid on old slides by electron microscopy? The neuro says no repeat on the lip biopsy as they are too painful and would not be worth it. (Fine with me) I remain seronegative on all autoimmune test, even the very highly specialized neuro antibodies, celiac disease, etc. Positive PET scan in one area, being 'watched'...if you PM me I will talk more, but, don't want my whole medical history on here. Don't know if amyloid shows up on PET.

[glenntaj]

--show up on PET--the PET scans measure oxygen usage rates that would not necessarily be elevated/reduced in amyloid-containing tissues--but, possibly, some effects of amyloid in tissues might be interpretable by a savvy PET investigator. (I suspect that a lot of these potential effects could be explained by conditions that are far more common, though.)

It might be hard at this point to use the old lip biopsy samples to test for amyloid--generally a fat biopsy (stained with Congo Red, to reveal the monoclonal light protein chains) is considered a more sure way to go (although almost any body tissue can be examined). The reason fat biopsy is often preferred is that amyloid tends to congregate in extracelllular spaces and that is relatively easy to see when aspirating fat cells.

Amyloid--both the acquired and familial types--is very rare. One would think that there might have been some indication of it previously if it were there (though it is true that doctors don't think to test for it often). It can be associated with sicca syndrome, but there are a lot of other conditions--especially seronegative Sjogren's, which can also mimic the neuropathy of amyloid--that seem more likely at first thought.

Have you ever had an immunofixation electrophoresis of both serum and urine to check for M-proteins or Bence Jones protein fragments? Almost always people with acquried amyloid have some degree of monoclonal (M-protein) presence:

http://www.neuro.wustl.edu/neuromusc...er/amyloid.htm

As you may know, Kmeb is our resident expert on amyloid testing/biopsies (though to my knowledge she hasn't has a lip/salivary gland one done that looked for that specifically).

PM us, if you want, with other info you think is relevant.

[BEGLET]

Hi,

I've been reading this - and I'm going to try to find my slides - I had 5 salivary glands taken out about 5 years ago and they were looking for Sjogrens - I need to dig out the results and see if the slides are still around... cause, my docs are again looking for amyloid....

I'm having intensive cardiac testing done - because I now have cardio-myopathy affecting 3 valves, ejction fraction down, waiting for resuts of test from a couple days ago and have more cardiac testing next week - but 3 cardiologists tell me it looks like amyloid... I've had a positive and negative fat biopsy - they are only 50% effective at detecting the disease, people often look for years for this and then finally find after biopsy of many organs even with normal bloodwork... my blood tests are ok - but my docs are still very suspicious and after the cardiac testing is done they are going to do complete GI testing for amyloid with lots of biopsies... this disease can unfortunatey hide in any organ...

I guess if all the slides are already stained from the salivary glands -I believe for amyloid they have to stain with congo red - they would have to redo (thats biopy that HURTS by the way)... would love to know if anyone has the answer!