Have I got this right?

[MAT51]

This is a question that has been bugging me for a while. Am I right in thinking that Sjögren's is principally a neurological AI disease in the sense that autonomic dysfunction lies at the heart of it? Or is the autonomic dysfunction only one of many neurological and glandular manifestations?

I suppose the reason this question keeps surfacing over and over for me is due to the fact that I have  been raking through my clinical notes and letters for purposes of applying for PhD funding - and for a disability benefit that is supposed to help people to remain or become more independent in their daily lives. From the PhD aspect I'm applying for funding to research systemic fatigue and other sensory disturbances through the eyes and practice of an artist with lived experience. This is going to include speaking to Sjögren's experts and so it really is important to me that I, as a non scientist with lots of fatigue and some cognitive impairment - gain a better understanding of some of the more rudimentary  mechanisms/ biology occurring in Sjögren's and other AI diseases.

In raking through these letters I realise how many times Sjögren's was ruled out, or thought highly unlikely for me because I have plenty of spit. I now have my dental hospital records and, in the same week that my parotid ubltrasound was entirely normal "showing no evidence warranting further investigations for Sjögren's" - my lip biopsy showed that the only four small salivary glands they could find were 3mm long, brown in colour and each had >50 clusters. There were also clusters of plasma cells. Overal conclusion being "meets all the Cheesolm's criteria for Sjögren's Syndrome". So why would doctors, dentists and max fax surgeons and 2 rheumatologists have excluded the possibility of Sjögren's for me for years on the strength of Sicca being too mild if it has infiltrated my saliva glands so decisively I wonder? Are we still not even at base camp in our understanding of this disease perhaps?

And still my mind turns over and over on this point of believing the person with lived experience first and foremost. How many of us actually suffer from the degree of somatosisation (sorry if this isn't actually a word?!) that many doctors seem to presume, despite knowing very little about us or have any insight into what we can learn from others on forums such as this along the way? Why can't rheumatologists and neurologists listen to the bizarre accounts many of us give of symptoms that would take a highly fanciful and imaginative person to make up - at face value? Why do so many doctors choose not to believe us? 

[Joe S.]

Now my memory and understanding could be wrong, but I tend to believe that Sjogren's is a disease that attracts all of the glands that produce a fluid in our body. (Lupus attacks the organs). At some point during the illness, our autonomic system is impaired. This impairment can be from the glands not functioning properly.

Having said that, I believe that in auto immune diseases nanobactria and mycoplasmas attack the mytochondria DNA of our cells; damaging those cells and causing our immune system to attack them. This would be a normal function of our immune system.

[MAT51]

Thanks Joe. Doesn't the area of the body that comes under initial attack depend on the type of Sjögren's a person has? I'm not good at the T and B cells thing at all -but if you are right about this then might a person's body have worked it's way through the glandular attack and response long before any doctor makes the connection - and then start working on destroying other parts, including the nervous system and organs? I was told by some one here that even Sicca is caused by a dysfunctional nervous system. I've also read on John Hopkins and Oxford Rheumatogy Journals that seronegative is different in that the sufferer is much more likely to have nervous system involvement and those who are seropositive are more likely to have lymph node involvement.

Now I need to try and apply my fatigued brain to understanding your second paragraph!   

[Joe S.]

I am Sero negative still according to my blood work but then I have been taking supplements to repair damage to mitochondria DNA (R-lipoic-Acid and Acetyl-L-Carnitine). If you have one AI disease you tend to get more of them.

So, if it is from nano bacteria and/or mycoplasmas and it is an error that requires two of these to cause the damage like a two part poison. My research suggests that there are 16 nanobacteria but I have no idea of the number of mycoplasma that are available for this process. I once had a list of what nanobacteria can trigger which AI disease. I lost track of it through the loss over various computers.

One article suggested that Gene 6 & 7 were damaged in the telemires (sp).

[MAT51]

Sorry Joe but my brain just will not function where numbers and science are concerned unless these are part of a process I can relate to personally. And even then I struggle with some aspects of my own bloodwork such as thyroid stuff. I know stuff like my +ANA numbers as I know what's elevated in my Cellcept monitoring and my CRP and PV/sed rate but that's it. I'm a very amateur sleuth indeed!

[cccourt1942]

Hi,
   This explanation for SjS is on one of the sub topics on this site.
 
    Basically, an autoimmune disease is when the immune system turns on one's body and attacks particular parts or organs.  In SjS the B cells attack our salivary and lacrimal glands: specifically the mucous.  The salivary glands are rendered sludgy to atrophied.  If you are not diagnosed, and the disease progresses, those glands can be damaged irreparably.  The absence of mucous to one's eyes results in retinal damage...but all we feel are anything from a speck in the eye to a stick in the eye!!  Restasis reversed the damage to my coating, but did not restore tears due to  atrophied lacrimal glands.
    I was not diagnosed until 71.  I suffered various symptoms for about 25 years.  The REAL discomfort started about 5 years prior to dx. 

Other damages can occur, and do, but saliva and lacrimal fluids are most frequently observed. 

Because of the effects of absence (or decrease) in tears, your autonomic system is affected as blinking is an autonomic response.  The same goes for the effects of decreased to zero saliva production:  if there is no saliva, it becomes difficult to swallow (another autonomic response) and so forth and so on.  This holds true for other areas of one's body which Sjogren's can affect.

This is a simplified explanation.  Look for the sub topic which says something "more about Sjogren's"
Best luck to you on your work,
ccc

[MAT51]

Thanks Cccourt. This is probably the best explanation I've read for the autonomic dysfunction. My eyes have been dry for as long as I can recall.

Would you also be able to explain to me why my mouth might feel dry when it actually isn't? Are my autonomic and peripheral nervous systems overreacting to minor dryness that dentists and oral consultants are unable to detect perhaps?  All the dryness seems to be in my nasal passages, throat and respiratory tract - and probably all the way down into my colon.

And yet the front of my mouth appears to have plenty of saliva I'm told. But perhaps it's quality rather than quantity - as my optician tells me. My worst symptoms these days seem to be the rancid taste, the icy SFN in my arms, legs and face and relentless tinnitus, loss of smell and numbness and tingle in my face. I'd trade these for worse Sicca symptoms readily - even though I often struggle with choking and swallowing just now. I'm getting a bit lazy about my eye drops but they never burn or hurt - just feel a bit blinky, sticky and annoying as they have for most of my life. I use a great gel called Artlelac and Hyloforte drops. Dry eyes and throat/ larynx certainly do contribute to the awful fatigue though. 

Maybe some of us have a more neurological form of this disease than others. I've certainly never had damage to my eyes or a swollen parotid and nor do I suffer from swollen glands. Maybe it's the difference between seropositive and seronegative as this paper on the systemic burden of Sjögren's suggests? https://academic.oup.com/rheumatology/article/55/suppl_1/i179/1795516/290-The-Burden-of-Systemic-Disease-in-Patients?searchresult=1

[Carolina]

Sjogren's is primarily a neurological disease.  The system that produces moisture is an autonomic nervous system (Parasympathetic).   Your autoantibodies attack this nervous system and damage it.

So you have dry eyes, dry mouth, dry everything.

Sjogren's may attack any organ or system in the body...but most often it attacks the nerves, causing peripheral neuropathy, small fiber neuropathy, even carpal tunnel disease. 

Read the information at the beginning of the Sjogren's Foundation page.

http://www.sjogrens.org/home/about-sjogrens/symptoms

At the point I have all of the symptoms.  The symptoms may not seem neurological (dry mouth) but it is an attack on a system of your body that has a neurological function.

I have reduced hearing, lung dysfunction, IBS, Interstitial  Cystitis, Profound PN, Terrible Small Fiber Neuropathy (the skin on fire disease), Dysfunction of my esophagus for swallowing, and on and on and on.  I am completely disabled.  I take many medications and supplements to manage my pain. 

All caused by attacks of my Immune Disorder on my Nervous system.   I also have an immune deficiency, and must have IVIG every four weeks to keep from getting chronic/life threatening infections.

Ain't we got fun?

Regards,  Elaine

[anita]

Sjogren's is a rheumatological disease...and classed as such.  Although the glands are a part of the parasympathethic function the disease process is connective tissue and effects this function.  That doesn't make it the cause of the disease process.  If it was a neurological disease, is would be classed as one, and it's not.  There are MANY neurological manifestations of the disease...effecting brain, spine, etc, but this attack is not common in all cases.  Actually, most patients still just have dry eyes, mouth, etc while other have severe neurological damage to both the autonomic and peripheral nerves (autonomic being a subset of the peripheral), as well as brain damage (Central nervous system).

As you know, Mat, I had neurological manifestations BEFORE I had the profound dry mouth.  I always had some mild dry symptoms, but never complained because it wasn't severe until a couple years AFTER the neuro symptoms hit.

Hope this helps.

[MAT51]

Yes Anita - both you and Carolina have helped a lot. I did no science at all at school and am dyslexic so I find it quite hard to process information unless I can visualise it in allegorical form. So I need the story to be spelt out for me in exactly the way you have in order to draw it out in my mind - and then later onto canvas or paper.

For me the worst now is the icy wind blowing through my legs and the constantly freezing feet. As the temperature has dipped to autumn I seem to have the burning pain back in my finger tips and under the distal part of my nails again - this time in all my finger tips and visibly manifesting as splinter haemorrhages in my big toenails. This and the numbness in my face, awful rancid taste and burning lips and tinnitus all persist but the tendinitis and synovial joint pain have all cleared off since I started the highest dose of Cellcept. I don't believe I have a somatotisation disorder of course so keep wondering if the C6 root degeneration spotted 3 years ago on MRI and the significant wear and tear arthritis in my lumbar region might not be behind the persistent sensory issues - which would explain why Cellcept isn't helping these? I have had nerve conduction studies done last year and nothing showed and my neurologist therefore puts everything down to the SFN but could the osteoarthritis still be responsible do you think it am I just trying to find mechanical reasons where all is actually SFN?

My CTD doctor and the neuro cite the improvement in disequilibrium as the main reason for me to continue taking the Cellcept - and the fact that I'm tolerating it so well. But I wonder how much is really improved and how much we just adapt to over time? Feeling slightly drunk may have just become my norm so I don't notice it much anymore unless I'm hitting the crumple fatigue zone.

Certainly, for me, the worst things of all are definitely the rank taste, swallowing issues, intermittent hoarseness  and the numbness and tingle and sensation of pressure in my face, the fatigue, the cold legs and feet and consequent collapses - when I can't cope a minute longer - sometimes in the middle of the street. And the fact that when I tell my health professionals about these symptoms they describe them back to each other in clinical letters as if I was just being too sensitive -  using terms such as myofascial pain and arthralgia with "functional??" occasionally to be spotted! I don't really believe myself fanciful enough to make this horrid stuff up and I don't think it's myofascial pain at all - I think it's all due to neuropathy of one sort or another - cranial, SFN, arthritis induced - whatever.

And furthermore they can't expect to tell me that they will be led only by my account of symptoms progressing or improving with Cellcept - but then imply I'm being too aware and introspective of these multiple symptoms. Feeling rather exasperated but hey ho. I'm actually emerging from a 3 month flare up of arthritis/ sciatica and fatigue of a new order - so an feeling frustrated and cross with my medical team - neurologist and oral consultant particularly. This is probably no bad sign as haven't had the energy for irritation/ exasperation until now!

Take care Anita and Elaine - I know from your accounts that things could always be very much worse so I try to count my blessings when I remember to! Xx

[Kristian]

MAT51-  Have you tried Exovac or any of the salivary gland stimulators?  I had a horrible time talking because I would get so hoarse I couldn't continue and would have to stop eating because I could no longer swallow.  Exovac has made a world of difference for me and would be worth asking your rheumy about if oral dryness is your biggest issue.  Keep in mind it helps big time but definitely not back to normal functioning.

On the thread, I think the disease affects neurological systems after it starts, but is not primarily a neurological disease.  IT first starts by reducing function of moisture producing glands, whereas purely neurological would reduce the messages sent to moisture producing glands lowering their production (autonomic neuro-system).  The disease definitely goes after the neurological system as it progresses more as more than few doctors had me pegged for MS before Sjogren's was agreed upon.

Hope everyone is well, very interesting thread.

[MAT51]

Thanks for this. To answer about Exovac - this isn't available in the UK where I live but Polocarpine is approximately same I think - however there's been a big supply issue abc many haven't been able to get hold of it for nearly a year now I'm told. I did ask at my last CTD clinic appointment but the Scleroderma doctor I saw said that not one of his patients has liked or tolerated it yet. As I've had many very severe drug reactions I'm wary - even if it was being offered to me which it isn't just now. I think I've probably had Sjögren's for decades - since I was a kid even - and I was diagnosed as hypothyroid when in my late 30s. So my teeth were terrible as a child and younger adult and I had chronically dry eyes for as long as I can recall. And the only four salivary glands they could locate to extract were brown and infiltration of lymphocyte clusters and plasma clusters was 100% so I'm guessing this confirms my theory that I've had it for a very long time undiagnosed.

But now my eyes are just normally tearless and my mouth is only dry at the back and my teeth have stabilised, no serious gingivitis and just neuralgia sometimes from two very large root canals I had when young. This is why dentists and oral consultant were all amazed that I had such a difinitive positive for Sjögren's last year. I wonder if sicca sort of burnt itself out after decades of causing my oral decay and now is targeting my nervous system as the Sicca moves down into my larynx and attacks my nervous system instead? This is how it feels to me at any rate. Glad the post is of interest to you. Sometimes I post and feel I'm just going round and round in ever decreasing circles!

[cccourt1942]

Would you also be able to explain to me why my mouth might feel dry when it actually isn't?...All the dryness seems to be in my nasal passages, throat and respiratory tract - and probably all the way down into my colon.

I am not a doctor.  My responses are personal or that which I have learned thru doctors and reading.  To answer these questions I am drawing on my knowledge from same:
BELOW THIS you state your mouth is "wet" in the "front".  This means all those hundreds of tiny salivary glands are functional.  Mine are too...well somewhat.  My rheumy checks behind my lower lip each time I see her and invariably she comments "dry."  Well...to me it doesn't feel dry.  I guess it's relative to what I feel in the remainder of my oral cavity.
AND.......TO THE OTHER QUERY:  Heck if I know!  The same thing happened to me.  After decades of sinus infections, aggravations (including sinus MRI, about 5 years before my dx, they stopped.  To me, at the time, I thought of the congestion to my head (sinuses that is).  Not until AFTER dx did I learn I was dry in those other areas you note--and all the way to my colon!  I'd been given Prilosec (long before Nexium) about 20 years prior.  I said I didn't have GERD.  The gastro insisted.  NOTHING was ever found in a colonoscopy or endoscopy for years.  I had a chronic "clearing of throat" to which colleagues diagnosed me with a tic.  (Speech therapists).  Started the pilocarpine---and almost all disappeared.  It wasn't overnight.  Had to adjust to the meds.  That is, took me  a long time to work up to full dosage.  Pilocarpine tore my
stomach up.  After nearly a year on the pilo and plaquenil, I started on LDP (low dose prednisone). This is the drug which helped with me with everything I BELIEVE.  I know it is as when I take more I feel spectacular.  I am allowed to up it when I travel.  When I decrease it, I am a wet noodle...and hurt!

... icy SFN in my arms, legs and face and relentless tinnitus, loss of smell and numbness and tingle in my face.  I HAVE THE HANDS...and have had in my feet (but only following sprains or other injuries) but it has not spread.  But the tinnitus?  I keep music going constantly.  I keep the TV on all night (low) for white noise.  I'm seldom bothered with the tinnitus with those interventions.  Again..the prednisone has to be the key.  It used to be very bad.  I lost smell AND taste for several years.  Smell continues to be minimal, but taste is back.  Again: I attribute this to prednisone.
PROBLEM WITH PREDNISONE:  you are young.  I am old. I won't live much longer.  They won't give this to you at your age. 

I'd trade these for worse Sicca symptoms readily - even though I often struggle with choking and swallowing just now.
SICCA or SJOGREN'S:  The DSM (in America) removed Sicca since I was diagnosed.  My dxing rheumy drew my blood as he said I had sicca.  I didn't care as long as there was a solution.  But I WAS positive.  That sicca will bother you with swallowing...and when you have no saliva going in your oral cavity, you have none in your esophagus.  Water literally washes out any good saliva.  I am not the only one who uses creams, dressings, dips (etc) when eating to help coat the esophagus.  As to the choking:  for about two years I would wake up "coughing".  Not until I learned what was wrong with me did I realize I was choking:  No way to swallow (autonomic==remember).  I use a sip of water and a squeeze of biotene gel in the night to keep everything moist.  It helps with the teeth too.
                I hope I answered these questions ...again ...looks like a lot but attempted to be brief.
ccc

[MAT51]

CCC - yours and other replies are SO helpful to me  - many thanks. I think it's hard to quantify the severity of what we each suffer because, the longer we live with our symptoms, the more we take them to be our new normal and even get used to them. So we may assume things are better - or not as bad as what others deal with - but Sjogren's is not only invisible - it's impossible to assess objectively for disease activity. With my previously misdiagnosed RA - they used my Sed rate and other bloods to monitor my disease activity. But now I'm told that Sjogren's inflammation is uniquely untreatable and these markers do not show active disease with Sjogrens, as they do with Lupus, Vasculitis and RA. For someone who depends on visual clues - including print out of blood results - this comes hard. I don't find it easy to know how bad things are all by myself - but this is what my doctors tell me they will be guided by. And yet when I attempt to list the symptoms that have improved, stabilised or worsened - they imply I'm over thinking and overly aware of multiple symptoms. There's no winning here it seems!

My view of dry mouth is that, for many with Sjogrens, it's the quality of saliva and tears that matter far more than localised dryness. Those having chemo or on antidepressants often have severely dry mouth - whereas friends of mine with Sjogrens usually say the same thing as we are saying here - front of mouth is often okay. But nasal passages, throat, larynx, oesophogus etc can be like sand paper - or "as dry as Ghandi's Flipflops" to quote someone on an RA forum the other day!


Yes Prednisolone made me feel great too- although I put on tons of weight and had severe gastritis from it too. It masked all my bloods and pain and even my eyes and mouth were less dry so Sjogren's was ruled out. I pleaded with the CTD doctor to have steroid shot for arthritis pain or get back on a low dose of Prednisolone again a few weeks ago. But he was having none of it for the obvious reasons!

[Carolina]

OMG MAT I was reminded of the horrible taste in my mouth by your post.  Everything you are experiencing is like my small fiber neuropathy and the PN, as well.  Yes, tinnitus, which can drive a person mad.  I have a 'sound machine' next to my bed so that I can blur the tinnitus with a calmer sound.

On and on and on it goes.  Is it neurological or what?   Here is John's Hopkins on the neurological complications of Sjogren's:

https://www.hopkinssjogrens.org/disease-information/sjogrens-syndrome/neurologic-complications/

I am so used to the odd smells (wet paper, smoke?) that I experience from time to time, but the horrible taste in my mouth is hard to adapt to.  And I also have mild cases of thrush now, from time to time.

I guess we get used to most things as long as the pain is managed.  Of course.  And we try to forget that we once skied, or rode a bike, or went ice skating, or even for long walks and hikes. 

I don't feel self pity, however, since I can see the sun and enjoy the people in my life, and laugh. 

Regards, Elaine