Immunology has shifted to positive - accessing new meds possibly?
 

After five rollercoaster years of a diagnosis of non-eorosive, seronegative RA - my immunology has at last yielded clear positive results. I've had to be allowed to become rather unwell for this to happen but my ANA is now a clear positive, elevated IgG and IgA - CRP has risen slowly from 2 last year to 19 and my PV is 1.98. I'm to have a lip biopsy and ultrasound of salivary and carotid ducts over the coming month.

Meanwhile my rheumatologist has expedited my next appointment to 22nd August. I'm hoping this at last brings an acknowledgment that my SFN and possible Dysautonomia are part of an inflammatory process that needs modifying again. I have already tried for DMARD medicines and am severely allergic so it does mean that options will be limited even if I'm rediagnosed at last with Lupus, Sjogrens or UCTD. But turning gradually more and more numb - to the point where my mouth and left nostril and eye feel permanently anaesthetised - doesn't feel like a good option. I'm feeling permanently tired and vaguely disorientated/ dizzy because of proprioception issues now. I eat very well, research my GI issues and have tried eliminating gluten and dairy to no useful end. I have followed my new neurologist's advice and got back to simple yoga and gentle aerobics. But my balance is still off despite attempts to retrain my brain.

Two neurologists have so far shrugged it off as mild SFN and have felt it unrelated to an ongoing disease process. I am therefore seriously hoping they are now proved wrong.

Several of the members of this community have helped me push for answers more than words can express - I think you probably know who you are so please know how grateful I am. Onwards and upwards!

It's odd to say I'm 'happy' about positive results...but in your case, it means answers!!

First, I must ask what was the actual result for ANA (the number) and pattern?

The number tells how high it was, but more important is the pattern. There are four different patterns that are typically associated with certain AI diseases:

Homogenous---Lupus and mixed connective tissue disease

Speckled---Lupus, Sjogren's, Scleroderma, RA, and mixed connective tissue disease

Nucleolar---scleroderma and polymyositis

Centromere---scleroderma and CREST

At least now, testing will move forward...and therefore, more answers. Your pushing and yet patience have paid off.

Hello Enbloc. I can't access my results so don't know the breakdown yet. But I'm guessing, from the fact that I've been referred for a lip biopsy and MRI of carotid and salivary ducts ASAP - that it's either the Homogeneous or Speckled category. The numbers she told me were 1-320 and she said that this is non specific but with raised IgG and IgA and the high inflammatory markers and SFN - it would be highly advisable that I have the lip biopsy to rule in or out Sjogrens decisively. She said that it is possible to have Sjogrens without much mouth dryness and only mild Sicca. But without a positive lip biopsy result I'd be unable to try anymore immunesuppresant medications (and there are still a few left apparently) unless my RA symptoms return once more. Hope this explains.

Ps to Enbloc: I managed to get a copy of the rheumatologist's clinical letter and he only says that my ANA is positive 1:320 but the rest of my ENA is negative - no pattern outlined. Meanwhile my IgG is 13.80 (5.50-13.00) and my IgA is 4.37 (0.50-4.00). The PV and CRP are both high and my RBCs are high too - these three have almost always been raised to high though.

Certainly, they checked the pattern...it's standard to do so. He may know and just didn't mention in his report.

But, it sounds like they are pushing forward to test for various things...which is good.

How soon will these be done? When do you see him next?

I spoke to him on phone today. Unusually for NHS rheumatology he's brought forward my consultation to 22nd August - two months after my first one with him. He will have lip biopsy and ultrasound results back by then. I fear that I will be abandoned by them if this shows negative though. I didn't ask for a verbal breakdown of my ENA pattern. His English is quite hard to understand and it wouldn't have meant enough to me.

My GP was thinking my high inflammatory markers might be tracked down through yesterday's colonoscopy, but obviously not. I have been told by several people on Sjogrens helpline that SJS is not commonly associated with a high PV and CRP. And the rheum makes it clear that seronegative RA would onlynvery rarely cause SFN. He thinks Lupus would have caused kidney and lung problems by now as I'm post menopausal. I admit I'm still very anxious about Multiple Myeloma now I've read my blood results -including the raised RBCs. Heck why was I born such a worrier?!

Hey Mat,
Worry is something that, at times can be helpful; but you know too much can add some very unwanted stress.

You did manage to have some worry removed with your colonoscopy turning out well. As you know; that is a real plus.


Gerry

Gerry, my worry was never about having bowel cancer because I do trust my instincts and these told me that I only had a couple of haemorrhoids. I just knew that in my case, the colonoscopy would be unlikely to give me the clues about what is wrong. I also felt that I didn't have an IBD.

It is much harder to diagnose MM (I have a friend with it) and lots of my signs and symptoms point to it. But my instincts say that I might well have Sjogrens - hopefully the lip biopsy will yield positive results at last and then I will feel as if I've won the lottery! You can't hold worry down in a born Worrypot like me!

You are right though about knowing too much. But when two rheumatologists both run specific tests then inevitably I wanted to know what these tests were for. The first time I checked out the Bence Jones and sighed huge sigh of relief when it was negative (5pm Xmas Eve). By the time the second one showed negative I had learned that only 50% of those with Multiple Meoloma have positive Bence Jones. Finding out that I have Sjogrens or Lupus or my RA comes back will feel very good - but if not then my knowledge will have me pushing harder than ever for answers and that won't be a bad thing. Mat X

Mat,
You had mentioned why were you born such a worrier.
I was referring to worrying too much can lead to some unwanted stress. Not knowing too much leading to unwanted stress. Sorry for the misunderstanding. Of course you want answers.




Gerry

Have they done the skeletal survey...basically an xray of every large bone? This is standard test for MM and what they did for me since I have MGUS and positive light chains in blood, and therefore at higher risk for multiple myeloma. Of course, there is also the bone marrow biopsy...that is a definitive test for MM.

I think if they thought it was MM they would be doing this tests (bone survey and biopsy ASAP).

Mat, I agree with en bloc.

Those procedures were what happened for a friend of mine. Sadly, a multiple myeloma Dx was confirmed for her.

I think they want to rule in or out Sjogrens first Enbloc. No I've had little imaging done to date, apart from two chest X-Ray's last year and hands and feet for signs of erosive damage from RA. But I did have pelvic X-Ray done at the rheumatology clinic three weeks ago but I think this just showed wear and tear arthritis in hips and lower back. My ribs are really sore but I think they would have been covered by the chest X-Ray. My knees and shins are the worst for this bone pain but have never been X-rayed. I think and hope you are right that they would have checked. I'm not that confident in doctors though - the NHS is very hard pressed and things can easily just slip by. The new hospital does seem very thorough though.

Most of the time I really don't think I have MM. I don't have the light chains in my pee - they've done two Bence Jones tests this year. My GP thinks I have a rheumatic disease but they just haven't tracked it down yet. I'm crossing everything that my lip biopsy flags up Sjogrens as that would really feel like I'd won the lottery - sad as this may sound. Otherwise I worry that slowly I will be devoured by this numbness. It's as if I have had a local anaesthetic everywhere but in my trunk - or like I'm turning painfully to stone. The pain feels as if it's deep in my bones and nerves - especially in my knees, shins ankles and feet - hands too - and only affects me when I'm resting - otherwise it's felt as loss of sensation. Does this sound familiar to you or anyone else? It's gone on for so long that I almost can't recall it not affecting me now. Maybe for five years. I thought it was RA and so did the neurologist but my new rheumatologist says most unlikely with seronegative RA. My lips and gums and left face feel so like they are half anaesthetised constantly.

One extra thing. My red blood cell count is always a bit above top of range. Do you know if this is significant re the SFN? I googled this but it just says dehydration - however I drink water like a fish!

For what it's worth, I have SFN along with deep bone pain in my hands, feet and sometimes elsewhere in larger bones in my arms and legs. I also have deep muscle aches and soreness. My SFN is still of unknown etiology.

Im with you immune sister!
 

Hi MAT,
I have posted here before. I just had a lumbar puncture which showed two paired Oligoclonal bands (not in serum, just spinal) AND was diagnosed on nerve biopsy and EMG with a dysimmune neuropathy - basically axonal and demyelinating. He is calling it a sensory version of CIPD. I initially came up on serum as positive for paraneoplastic antibodies but they were not positive on western blot; however, that test is still pending on my spinal fluid and we will get them back the end of next week. I did have a negative PET/CT scan.

I have a positive ANA, homogenous and speckled, but very low (1;40). I also have Raynauds and anti cardiolipin antibodies which have run the gamut from relatively high in the last ten years (70's range to negative, which they are today - check me tomorrow it might be up again, lol). I also have low serum complements c1q c3 and c4 which are usually associated with lupus.

I went to a very well known neuro research specialist in NY and the best we can come up with is a DYSIMMUNE syndrome - basically, SOMETHING got to all three of my systems. I have the CNS oligoclonal bands, I have failed autonomic tests and now this peripheral stuff. I have tons of cranial issues too (pulling sensations, eyes and throat burning, eyebrows dropping, face twitching, weird face movements). I also have a very high anxiety level -as the dr explained, the CNS is trying to fight an antigen or antibody so thats not unheard of to have psych issues.

I feel very lost that we cannot find this offender. It is ruining my life. I truly believe this is paraneoplastic because I literally "broke" over the course of three months in late 2013- with seizures, headaches, the neuropathy starting, etc. But yet I have had weird immunological markers for a decade before as well as a small neuro episode very similar 15 yrs ago so maybe it was building and building for a long time (unlikely to be paraneoplastic that long). We are going to try IVIG in the next few weeks as well as an IV of antibiotics for LYme because I definitly have exposure in my serum. It wasnt so clear in my CSF.

Anyway, I just wanted to let you know I UNDERSTAND on the spacy, foggy, not yourself and "this is definitely more than just a neuropathy" I am actually seeing yet another rheumy on Monday. I was wondering about sjogrens myself. THis is crazy. I can't believe no one can figure out why my immune system is attacking itself all over!!!!!

Best,
L

Oh this sounds really hellish - poor you of course I can relate. I've been researching more because I'm preparing myself for a negative result from lip biopsy. My SFN didn't show up in skin biopsies taken from my calves over a year ago - and I can't quantify how much it has progressed or not since then. I need it to show up in order to qualify for further treatment such as IViG or Rituximab. What I find most depressing presently is the amount of pain in my feet and legs again. This stopped for a while - apart from tingling numbness. Now the pain is back full throttle and encompasses my knees now too. The only visible sign I notice is that my feet and ankles go a blue-ish purple with Livedo if exposed to air. Otherwise I have no way of knowing if the acid dunking sensation is real.

When my knuckles lock during the night and are painful so I can't move the duvet or pillow - and are swollen of a morning - I am reasonably sure this is my RA. This visual clue reassures me - and allows me to deal with the night pain better. But the horrible burning neuropathy makes me grumpy bad miserable. I hope my Sjogrens lip biopsy proves positive. My new rheumy says SFN is not part of seronegative RA. Which leaves me high and dry unable to access further treatments until either a vasculitic rash shows up and cryoglobulins are positive or else my RA comes back properly. It is sad to want these visual clues so badly I know - but I do nonetheless!

Good luck with IViG. Mat x

My immunology NEVER shifted to positive.

And then at the age of 68 I was found to have a DEFICIENT IMMUNE SYSTEM.

I don't have enough immune antibodies in my blood to protect me from infection. I need infusions of blood from donors, every month, to keep from having constant infections..

And my Duke Research Immunologist told me that probably my Deficient Immune System is ATTACKING MY ORGANS/SYSTEMS and damaging them.

My Immune System is probably using cytokines for this attack, but we don't have the tests to identify this, yet. "We use the tests we have, not the tests we need."

And OF COURSE I was dismissed by every Rheumatologist and Neurologist as having 'whatever' "idiopathic stuff". Go home and sleep it off.

I had so many idiopathic things that I felt like a "pathetic idiot".

By the way, the attack of our Immune System, by whatever means, causes INFLAMMATION.

And INFLAMMATION brings three horrors:

PAIN
FATIGUE
DEPRESSION.

Like clockwork they appear with inflammation.

I'm sorry you don't tolerate medications easily. I hope the newer medications to suppress your immune system are more tolerable.

I recommend "How to Be Sick" By Toni Bernhard.

Hugs, ElaineD

Ha!
 

"I had so many idiopathic things that I felt like a "pathetic idiot"."

Elaine that is awesomely funny...that comment will be reverberating in me for a while.

I don't understand the workings of the immune system well enough to grasp the difference between autoimmunity and immune deficiency - can they overlap? I'm full of a yeast infection just now and no matter what I try - natural or medicinal - it won't clear up?

My small fibre neuropathy (idiotic and pathetic in equal measure!) is flaring properly for the first time this year. It is worse than it's ever been before. I felt as if my entire length of legs and arms had been dipped in a pure acid bath all night. I'm in shock this morning! I've been building up to this flare for about a month but nothing prepares a person for this kind of onslaught of pain��������

Mat, this is very complicated.

Immune deficiency comes in various forms. One is Common Variable Immunodeficiency (CVID), which ElaineD mentioned in another thread. Although it is labelled as "Common" it is very rare, affecting about 1:25000 - 1:50000 people. It shows up as abnormally low levels of antibodies which means that people with it susceptible to infections and other health problems. It seems that mutations in a number of genes may be important in it but this is not clear.

Another form of immune deficiency is Severe Combined ImmunoDeficiency (SCID) which is also very rare, affecting about 1:50000 people. It has various causes and sometimes a bone marrow transplant can be effective for it.

HIV infection can also cause immune deficiency because HIV kills helper T cells, which are needed for the production of effector B and T cells, which are needed (in different ways) for dealing with infections. In Western countries HIV infection is seen as a chronic rather than an acute problem because of the availability of anti-retroviral drugs which stop HIV from replicating.

In general autoimmune diseases are a bit different. The immune system can recognise foreign proteins (antigens) made by pathogens (viruses, bacteria, etc). When this happens both T and B cells get activated and, in different ways, work together to destroy the pathogen. Usually the immune system ignores proteins made by normal body cells - in immunological jargon it is "tolerant" to them.

Sometimes this goes wrong. A protein antigen made a pathogen might, in a chemical sense, "look like" a protein made by normal body cells. When this happens the immune system damages the normal body cells which make that protein, "thinking" that those normal body cells are pathogens - this is an autoimmune disease.

I hope that this is not too long and makes sense.

Kiwi I will cut and paste this sink can digest it properly at my leisure. I'm very grateful to you for this fascinating information. Many thanks. Mat

Kiwi described it quite well. I will only add that up to 25% of people with autoimmune disease are also immune deficient. I am one of those with both CVID and Sjogren's.