Autoimmune Epithelitis

[inga]

I think this term is worth a google.  It is the alternative name proposed for Sjogren's.

If you google epithelial tissue, you will see how invasive an attack on the epithelial tissue of the body could be.  This tissue is located in many regions of the body, external and internal....not just 'glands'.  It is in the gut (mouth to rectum), the kidney (distal renal tubules), blood vessels, anything with 'mucous membrane' etc.  You will see that it has NO blood supply but only a nerve supply, which is interesting given the link to neuropathy.

Epithelium gets it nutrients by diffusion by connective tissue.

A disturbance in the epithelial tissue of any organ could cause profound effects on the body....for example, distal renal tubular acidosis can cause destruction of bone.  You name it, if the organ system has epithelium in it, or is dependent on epithelium for function, it can be affected by this.

This makes far more sense than trying to explain 'exocrine' dysfunction which is only a part of it.

As for how to diagnose it, I would think one would need serum markers OR a biopsy of tissue to infer systemic involvement, and justify systemic treatment with all the risks it involves.

[anita]

Interesting.  I pulled up one article that said the name, Sjogren's doesn't seem to cover the complete manifestations and most point back to the epithelium. 

My first question would be how to test (biopsy)?  Are blood markers even defined yet?  Would they be the same SSA & SSB? 

Back to biopsy...when they do a lip biopsy, could they biopsy the surrounding epithelial?  Am I even thinking of this in the right way...that the tissue surrounds the glands, organs, etc?

If so, then couldn't they biopsy the epithelial in many different places/organs?  It seems this would make diagnosis not only easier, but more precise.

[inga]

Maybe this link helps answer the question...It's kinda long and complex, but the last paragraph seems to sum it up.

http://docs.google.com/viewer?a=v&q=cache:XDuTVwR8l0MJ:sicca.ucsf.edu/LSG_bx_Grading_SOP.pdf+salivary+gland+biopsy+lymphocytes+periductal&hl=en&gl=us&pid=bl&srcid=ADGEESgqsJlm7DXhGA9OnrAYPaaL6_82TN9ng-0JY_U_YZhxhJKbMmvanO6eWD572GZiuUU2FjsxyitDpafeVMRNs8wL1CWsgJ7PRZj4eIGats4SD86Dx_ghI1PbJHbu7PBFkaFkXDyU&sig=AHIEtbTbWuzGG1E9E3TmDtmzBsw3-Rbx3g

OOH My, that is quite a web address!

[inga]

I think they do labial salivary since it is the easiest?

I think they will identify a number of antibodies not just SSA and SSB.....seems like Sicca comes with many AI diseases.

There is a big article on Sicca and lip biopsy in relation to MG.

I wonder too, if they could just do a needle biopsy of several organs and figure out if this is the culprit.  If so, Epithelitis would be rather serious if systemic, IMO.

[Sha]

Video (need subscription for full topic) but gives good details:

http://hstalks.com/main/browse_talk_view.php?t=196&s=196&s_id=23&c=252

http://www.google.com/m/url?ei=SY4TTfjUKoLmqQPxy5CAAQ&q=http://www.danskreumatologiskselskab.dk/tekster/orientering/SS-clinical%2520wo%2520links.ppt&ved=0CBcQFjAC&usg=AFQjCNHJCgK63wRHzCA43cJddkxF4P2H2A

http://www.google.com/m/url?ei=Oo4TTeDaAYmyrAOu-oqAAQ&q=http://www.danskreumatologiskselskab.dk/tekster/orientering/SS-clinical%2520wo%2520links.ppt&ved=0CCQQFjAG&usg=AFQjCNELf7igScVTIP-T7SzQaDcw8dKeUQ

http://www.springerlink.com/content/w851311246g07870/

http://onlinelibrary.wiley.com/doi/10.1111/j.1601-0825.2006.01292.x/abstract

http://onlinelibrary.wiley.com/doi/10.1111/j.1601-0825.2006.01292.x/abstract


YOU TUBE VIDEOS

http://www.youtube.com/watch?v=4RerelUHKF4 This gal has a lot of videos on the subject



http://www.youtube.com/watch?v=-PIcNX5SJ0s&feature=fvw

[anita]

Okay, this will likely sound stupid:  What the difference between epithelitis and FLS (focal lymphocytic sialadenitis) or FSLS (focal sclerosing lymphocytic sialadenitis)?  The epithelium is the tissue around the gland...not the gland itself, right?

Of note: my lip biopsy does not show FLS or FSLS.  They note that the acinar structure is completely lost and replaced by sialometaplasia elements and chronic sialofibrosis.  These areas are diffusely infiltrated with lymphocytes and plasma cells, but due to the structure loss there are no aggregations of 50 or more lymphocytes.  So although mine only "grades" at a level 2 on the greenspan (due to no foci), because of the completely loss of acini and degree of fibrosis, the findings are consistent with Sjogren's at end stage (their words).  There is no mention throughout the detailed report of any specific testing on the epithelium or surrounding tissue.  They also didn't test for Coxsackie involement (which would have been interesting).

I am certain there are other markers.  I actually give extra blood at each visit (at Hopkins) for testing/studies of new markers.  And yes Sicca comes with many things, but Sicca may not mean Sjogrens.

The needle biopsy is sort of what I meant in regards to precisely diagnosing epithelitis in a specific affected organ (if there is already an identifiable function problem).  This would help to define the involvement so treatment can be tailored specifically with better results.

The article you linked should be the one and only guideline used for lip biopsies.  

[inga]

Anita, it sounds like your salivary gland has been obliterated by something.  My neuro says the lymphocytes indicate inflammation, and that would be my take on it.  Your case, like mine, isn't textbook.  I am still not convinced by a lot of the 'diagnoses' we get.  My mouth is not seriously dry (eyes are awful, but I still make tears....don't know where they are going tho!)

As for the girl on the youtube video....I can't follow much of what she is saying....maybe it is me, but, I dunno....I did catch the words hypochondria, (which she said she is cured of, and klonopin.)  Great.  I appreciate people making videos on diseases, but.....The Greek doctor is good.  He does a lot of research on SjS.

This whole labial gland biopsy thing is up in the air....sure you find out if 'something' is going on in the gland....but, without more specific histology....I don't know.

There are so many ubiqutous viruses out there that could cause this.  If you want to read something disturbing, read up on Simian Virus 40.  SV40 for short.  I, for one, am fairly certain I was 'innoculated' with it, given the time frame.  I will have to look up Coxsackie and get more info on it.  Been a bit preoccupied with holidays.

I agree, they should biopsy more sites, or a site other than the labial glands.  I wonder if they could do the distal renal tubules or if that is too hard.  I would not want any one digging in my lacrimal glands.  Just for info....my nasolacrimal gland on one side is huge....has gotten blown way up...show up on CT as a big black hole.  This isn't seen often and the ENT didn't know what to make of it.  It gets air in it and I can play my eye like a musical instrument.

[shortstuff]

I am very much interested in this because I supposedly have a form of Interstitial Cystitis, the doc called it urethral syndrome.  Basically my urethra hurts/burns.  Others with IC have horrible bladder pain and are in the bathroom 60+ times a day.  I had a cystoscopy twice and both times my bladder appeared fine but there was redness/inflammation at the base of my bladder neck where it joins with the urethra.  The skin in this area is epithelial, to my knowledge.  I've had these problems since after giving birth to my 2nd child in fall 2007.  My eye problems started getting worse in 2003.  My mouth has been getting worse over the past year.  I have no doubt that it's all related and I now question whether I actually have IC or if IC/urethral syndrome is a symptom of Sjogren's or some kind of epithelial lupus.  A high proportion of women with IC also have Sjogren's.....it just seems to make sense.  I wish I could just be a guinea pig for Dr. Faustman. 

[anita]

I think Sjogren's use to be defined to just "dry eyes & mouth", then they started realizing the involvement was more widespread so it seems to have become a "catch all" for patients with a collection of symptoms that 'included' the dry eyes & mouth but now obviously systemic to involve multiple organs.  I guess this is where the autoimmune epithelitis comes into the picture.  It seems to define the nature of the disease a little better (at least for me).

Basically I think all autoimmune diseases are interrelated (hence overlap) and there is one component that triggers the antibodies to go nuts...then they head in different directions (Lupus, RA, Sjogren's, diabetes, Thyroid --or a combination).  

Although many have a hard time getting diagnose, I still think the label, Sjogren's, gets applied way too often for what is likely simple Sicca caused by something else.  

After reading many posts and details provided, I think there are several members here that i doubt really belong in the Sjogren's category.   I would go as far to say that I've questioned my own diagnosis at times.  My lip biopsy clearly shows almost complete destruction, but does that really mean it's from Sjogren's?  I have far too many neuro related problems that surpass the 'dry' problems any day.  And I'm sero-negative.  Sometimes, it's just doesn't make sense.

Inga:  I really am curious of the layman description of epithelia.  Is it just the 'covering' tissue?  Does it always 'surround' organs, glands, etc?   What about the in urethra like shortstuff described...that would be ''inside" the organ, so to speak?  Would the sheath or myelin covering nerves contain this epithelia?

This autoimmune epithelitis would make more sense (I think) than autoantibodies attacking nerves, organs, etc directly.  They would have to go through the epithelium first, right?

[inga]

I am posting this reference, since it really describes epithelial tissue well.  After reading this, it clicked in my head that if SjS IS REALLY an Autoimmune Epithelitis, as suggested, it does explain a LOT of the problems....and I wonder, since epithelial tissue does not contain any blood vessels for nutrients, it absorbs it from connective tissue, and since it DOES contain nerves, that does tie it to neuropathy and connective tissue disease, by anatomy alone.  If epithelium isn't getting proper nutrients for some reason, would not the nerves in it suffer similarly?  Just a thought?  (My mind is really a bit gone today, and one of these days when I am feeling a bit less 'holiday' stress, I will try to reason this thru.)

I TOO question what is going on with my body and diagnosis.  I do feel this diagnosis is too liberally applied to people and I fear that people simply will 'settle' for whatever name is tossed out there or be 'happy' when their misery is given a name, because it is hard to be miserable with no name to the malady.  Ten years ago, when I found the Sjogren's diagnosis, when I had dry eyes and other misery, I thought for SURE it had to be that!  I didn't want to hear that it wasn't this!  Now I don't want to hear it IS this!

My goal is to find the exact cause of this, so I can try to stop or remediate it in myself.  I know my cause is likely different than in others.  If the criteria are not exactly the same in research cases, the researchers will come up empty handed, as they have so far.  There are WAY too many conditions being labelled SjS and, I may be one of them.  I would venture a guess 50% or more of the posters on here are improperly diagnosed, and improperly treated, and may get damaged due to it.

I have always said, risk versus benefit on the drugs used, either to manage or to attempt 'treatment'.

[inga]

Anita, you had the high CD4+/CD8 ratio as well?  Right?

It's hard to keep all these details correct....pffflua..... the hard drive that was my brain is a bit full.

[irish]

Shortstuff, Bingo, I had the same thing for years. It is bloody miserable. Mine started when I was about 21 years old. The first time I was in nurses training in Rochester and went to Mayo clinic. They did the silver nitrate installations which I had to hold in my bladder for about 1 1/2 hours. I also had a urethral suppository of hydrocortisone and a product that I am having trouble remembering right now. I think it might have been called furacin. They don't use it anymore that I know of.

Both these things helped. They burned like heck and were miserable but they helped. I had to have that done a few more times. The area that is affected is the trigone. This is the little triangular area of tissue right above the urethra that tells you that you have to urinate. When it is inflammed (not infected really) it hurts all the time. Nights are terrible also cause your back hurts then.

I had that a lot and doctored off and on trying to find help where I lived. I had one doc who knew what it was and treated me with the urethral suppositories before it got bad and I felt better.

I had to go several other times to the Mayo Clinic and would have to go everyday for one week. I would have Argryol (sp) every day after they had dilated my urethra with what felt like a water hose. The dilatation was to massage the little glands in the urethra which emptied them of any possible bacteria. The last thing in the appt was the urethral suppository.

The last time I went I was dilated with a size 34 "sound" as they call the metal tube. This is big. I could feel the tissue, hopefully scar tissue tear when they did it. After having that done for the week (the size 34 was done on the Friday) I told hubby that is the last time I will be doing that cause they will have me peeing in my pants. I was afraid they would wreck the sphincter and I wouldn't be able to control my bladder.

The interesting thing is that my urine was always negative. Whe they would do the cystos they told me that it looked like a big scab right above my urethra and was more of an irritation/inflammation. I had learned over the years that taking the pyridium (turns the urine orange) helps if you can start it early enough. Doesn't cure it though.

Also learned that Naprosyn helped a lot. Aleve is the generic. When I finally saw my immunologist for the first time I mentioned this and he told me it was autoimmune related. For some reason I have not had it the past ten-15 years. I think the autoimmune moved elsewhere in my body.

The other interesting thing is that I think my mom had Sjogrens but was undiagnosed. She also had this bladder thing and suffered so much. It is really almost unbearable. I told my hubby that I walked around clenching my teeth, literally, because of the pain. It can really ruin life. Cranberry juice doesn't help, antibiotics don't help. THe suppositories did help but they took them off the market many years ago.

Mayo clinic has one of their pharmacies compound this for them only. Sort of unfair I thought!!! This issues is called Trigonitis and most GP's have never heard of it. I don't know that it is related to anything else. It might just be that area is prone because of the type of tissue. I also think that intercourse does not help it one bit.

Hope you can get things resolved. It might be that Plaquenil would take care of that problem. Plaquenil  has a good anti-inflammatory effect. Irish

[anita]

Inga,

Yes, the last time it was checked (a ways back) the ratio was increased, but there were no morphologic features of lymphoma.

I really think the neuro involvement is the key in my case.  I'm actually looking forward to an upcoming MRI of the brain...then neck and lower spine.  I have been unable to have one with the pacemaker (since 2004), but Hopkins has a new protocol for pacemaker patients to have it done.  Dr. Birnbaum just got me approved (after months of trying) for the procedure which he says will require a cardiologist and anesthesiologist to be in the room.  I don't know what it might show, but sort of hope it helps to put some pieces together.  There is lots of coordination to get all these docs in one room for this, so don't know when it will actually occur, but they're thinking in January.

There have been some very unusual labs over the years, to include positive cryoglobulins (several in a row---years ago). They are negative now though.

One recent lab (IMP-Impression Serum) noted the following text:  There is an IgG lambda monoclonal gammopathy

The next page/lab (IFE Serum) said:  One band of restricted electrophoretic mobility is present in the IgG lane with a corresponding band in the lamda lane.  The band is clearly evident and migrates away from the anode.

I was thinking of asking Nathan or Baklavas about this, because I have NO clue what it means (even after an online search).

I think you and I have many findings & symptoms in common...as well as our opinions that question our diagnosis.

[anita]

Oh, and back to my other questions.  Please tell me if my descriptions of epithelial tissue is correct.  If it is, then small needle biopsies of this tissue around affected organs will likely hold answers to many of our questions.

I'd be interested in thoughts from Nathan and baklavas on this too.

[Lily]

A reading of "epithelium" that I found describes this tissue as the covering of both internal and external surfaces of the body including its organs.
This could make it easier to do biospies at various sites?

Inga, your topic is great.  The discussions among you, Anita and baklavas are really stimulating.  This is the kind of thing I was hoping for at this site.  Many thanks for posting it.

Don't know if there's any connection, but read that the mumps virus is in the same family as Coxsackie.

'Bye.

Lily