StillHoping, thanks again for keeping the conversation going, and for all this helpful back and forth. I didn't know that a negative lip biopsy can still mean Sjogren's. I'll see about the other two antibody tests, as I've already tested negative for the ANA twice. I think I may have some degree of TN, but it's very minor, for now at any rate. I'll let you know when I have the biopsy done and the other blood work, but it may not be for some time, if I stick around in Canada.

It seems that you're still looking too. I really hope we get more answers soon. I'm also curious to see what improvement you have on your new treatment, if you go ahead with it.

Welcome and thanks, SylvieM. This thread is really great. StillHoping and En Bloc has been so helpful. I'll ask to see an ophthalmologist tomorrow when I see my GP, for sure.

I didn't know about false negatives on the lip biopsy. Perhaps that's what happened to StillHoping's sample? It may be worth doing the antibody tests and getting the lip biopsy to see what comes up?

There are false negative (and false positives) for any test...what matters is how often it happens and I haven't seen anything that indicates lip biopies have an unusually high rate either way. What does seem to be a problem with lip biopsies is lack of pathology experience with Sjogren's and them not using proper scales. I have seen the results from many pathologists (via other Sjogren's patients posting results on forums) and many don't even use a scale.

I do know that ANA and specific antibody testing can be positive when lip biopsy is negative, but this usually occurs at early stages when there isn't enough foci on the biopsy to warrant a positive result YET. A focus is an aggregate of 50 or more lymphocytes, histiocytes or plasma cells...and more than one focus (within a 4mm sq section) is required for a positive biopsy result.

David,

you should have the antibody testing done, but don't be surprised if they are negative, since your ANA is negative. It would be somewhat rare to have a negative ANA and postive SSA or SSB.

Sylvie,

Your unusual presentation and 'lag time' may be from exactly what you thought...triggering an autoimmune process. It is also possible your neuro symptoms presenting first is from this same trigger...because MANY with autoimmune disease start with neuro symptoms first, then later have the more typical autoimmune symptoms from the specific disease (MS, Lupus, Sjogren's etc)...I am one of these patients. I got sick with a severe upper respiratory infection (that was the trigger) which set the wheels in motion. It started with dysautonomia symptoms affecting my heart rate, BP, etc. it wasn't until months later that the fatigue, dry mouth/eyes etc all started.


David, I thought I read somewhere you had some fears about IVIG. I started using it in 1997, so, I have a bit of experience. It is relatively safe but needs to be properly controlled and administered by educated nurses/doctors...and what to look for in regards to serious side-effects. I would be happy to answer any questions you have about IVIG...including what can go wrong (I've experienced that too).

Hope this helps. Please pardon my spelling errors, as I stil don't have an working spell-checker at present and most sites don't provide them anymore. So I'm winging it...lol

En Bloc,

Your responses are always helpful. Thank you.

I will ask for the SSA and SSB and ensure that the lip biopsy is done properly. I don't have any issue insisting on things that I think need to be done, whether the physician is offended or not. I have been referred to a leading rheumatologist, perhaps the best in the country, so I think the technical aspects should be fine, but he is known to be an arrogant fellow, so I'll see what happens. Arrogance can also and often does lead to incompetence. We'll see...

I'm seeing an optometrist in January, and then possibly an ophthalmologist. The former will run some tests, and then we'll go from there. However, I learned that for my age group, and since I don't have Glaucoma or diabetes, I will have to pay and it's not state covered, so I may delay and ask to see an ophthalmologist, though that may take months. I may just pay the $125 and get some testing and imaging of the eye, which is what I believe they'll do.

I appreciate your offer to inform me further about the potential negatives of IVIG. I would like that, since I plan to ask for it but that's my only hesitation. I've heard in some extreme cases there can be liver or general organ failure, among other things. I'm sorry to hear that you've experienced some negatives of the treatment. I hope that has passed.

So you've been doing the treatment once a month since 1997, do I have it right? If so, how much improvement have you seen in over 8 years of this treatment? And have you done further biopsies during that time to see if there are any signs of healing and not just symptom relief? Perhaps I have this wrong, and you've only recently begun. But I'm curious what the course of treatment is, if it's healing (if not entirely curative).

Thanks again, and Merry Christmas!

I started receiving IVIG in 1997 and continued until 2007 (10 years), then restarted in 2013 to present.

All 'big gun' treatments carry risk...and IVIG is in that same class. IVIG is a blood product. It takes about 2000 donors to make ONE dose. But as a blood product, there is always a risk of rejection/reaction. It's foreign to your body, so measures are taken to reduce the impact on your system. Typically, patients will receive 'pre-meds' before starting their infusion. Tylenol, Benadryl, and IV steroids make up the usual cocktail. However, one of the most important things to do before infusion, is drink...LOTS of water/fluids to hydrate even over hydrate a little. And continue to hydrate during and after the infusion to help reduce the risk of reaction. These side-effects may start during the infusion (and stop if you catch them and reduce the rate), but may also hang around for a couple days after infusion.

The most common side-effects of IVIG are headaches, chills, flu likefeeling, weakness, low grade fevers and muscle aches. The headaches are most common and can be mild or severe. All most all of the side-effects are rate related and may go away if the rate of infusion is slowed down. So it's very important to communicate ANY side-effects you start to get during the infusion, so the nurse can adjust the rate accordingly to keep you comfortable.

It is possible for the headache to develop into something more severe...like aseptic meningitis. I ended up with a severe case os aseptic meningitis in 2007 and this is why I stopped IVIG for several years. It is a rare, but serious reaction to IVIG and my doctor was not comfortable (for several years) continuing treatment. It wasn't until I had repeated infections (from my immmune deficiency) that we decided to try infusions again. Being that i would be at high risk of another case of meningitis, we started very slow and slowly increased the dose over many MONTHS. We also added Zrytec to my pre-meds to help curb any reactions. So far, I have successfully received IVIG for 2 years without any problems!!

One other thing I should mention, is that once you use a particluar product (brand), then you need to stick with that one. Just changing products can bring on reactions as well.

Also, although you may think going 6 months without a reaction means you won't get one...this is wrong! Every dose of IVIG is made with different donors, so each dose can cause a reaction. I had received IVIG for almost 10 years (monthly) when I got aseptic meningitis...so clearly years of use (same product) doesn't make a patient immune from a reaction.

Ask questions, be informed and communicate ANY side-effects as soon as they occur. Most people toloerate IVIG quite well if given slowly and they are well hydrated. Rushing to finish is not the way to go. My infusion takes 7 hours. Some products infuse much faster (because the concentration is higher).

It takes about 6 months to really know whether it is helping, but you may notice improvement the first or second month.

Hope this helps.

I hope I didn't sound too negative about reactions. I just want to be informative so you don't have any surprises. By in large, most people tolerate it quite well...with maybe some mild symptoms for a couple days.

The first 10 years I had periodic headaches that lasted a couple days along with general run down feeling for the same time period. The aseptic meningitis was very severe (normal white cell count in spinal fluid is <10...mine was over 700) and it bought me a week in the hospital followed by a full month of recovery at home. Due to the severity, we stopped infusions for over 6 years...until I just had to have for my infections.

I really think you might benefit from trying it, but you may want to try other treatments first. But most of the other treatments are designed to reduce the pain, NOT address the rott of the problem. So you end up masking the problem not fixing it. Steroids work by reducing the inflammation, so in that regards, it's IS helpful. But IVIG goes to the core and can actually make a difference in the overall problem...especially if autoimmune based.

Good luck and I hope I didn't scare you away from keeping this on the table.

Dear En Bloc,

Please, you didn't scare me off, weren't too negative and so on. I appreciate the honesty, the detail, and all your help. When it comes to such powerful treatments, I can't imagine there not being side effects, and serious ones too. I was, of course, sorry to hear that you suffered with aseptic meningitis. But I'm glad you survived the ordeal.


In the end there's no way to know how a given individual will react, so for me it could be much worse, as bad as death, or it could just barely make a dent in me, negatively or positively for that matter. There's really no way to know and from what you've said it sounds like it could be all relatively smooth sailing and the hit me suddenly.

But what you say is exactly right. I really don't care much for masking the problem but addressing it and IVIG goes to the root. I wonder though, given what you rightly note, whether I should try steroids first? My neuro did mention Prednisone, if it turned out to be autoimmune, say Sjogren's. I'm not sure I fully understand the mechanism, but is the idea that the steroid will reduce inflammation and allow the body to heal more easily, ideally at any rate? So it knocks out the immune system, suppresses it, in hopes of reducing damage/inflammation, but also thereby allowing the body to heal itself, very simplistically put?

Also, I was curious to what extent these many years of IVIG have healed you. Have you had any followup biopsies, say back when, before the incident? Perhaps it hasn't cured you, but has it healed you somewhat? Or perhaps it stopped the progress? Originally I didn't realize that you had been receiving the treatment for sometime years ago, but now that I know, I'm curious whether they used any markers to measure any progress or maintenance, not that this is easy to do.

Finally, there other day I posted this, but have not heard anything back that addresses the question, so I thought I'd mention it to you, in case you had some knowledge here: http://neurotalk.psychcentral.com/thread230404.html.

Thanks again for this very helpful back and forth.

I have to say: well, damn! The side effects of Prednisone are many and seriously bad! Not sure I'd want to go on it.

It's really hard to say whether the IVIG has 'healed' me in any way. All I can report is the changes that have happened since being on it. Keep in mind that although I started receiving IVIG 18 years ago, it was started for my immune deficiency, so a much lower dose than typically used for neuropathy. I did receive a 5 day high dose course of IVIG at this time for neuropathy, but no follow-up at the higher dose.

I hope to have another skin biopsy in 2016 to compare with my last ones done in 2009 & 2010. At the time, my density wasn't too bad, but the morphological changes were significant showing segmented, tortuous and swollen fibers in most of the SIX locations of biopsy (my doctor did ankle, knee, & thigh on both legs). This is where I think I'll see the biggest changes and proof of the IVIG working or not. I plan to see my neuro/rheumy at Hopkins in Feb/March, and maybe I can get it done in April or May (all depending on how backed up they are). I honestly think it WILL show improvement, but I want the proof.

Let me tell you a little about steroids. Although they have a ability to reduce inflammation and allow for better healing of the nerves...this all comes at a hefty price...a life long price in some cases. Steroids carry even HIGHER risks than IVIG (In my opinion). I say this because the damage done by steroids can be permanent (in many cases) with damage to joints, bone density, skin, GI, diabetes, infections...the list goes on and on. Not to mention that steroids have just as horrific withdrawal syndrome as any narcotic that can take months if not years to get completely off the steroids.

I do, however, think a short course of steroids to see if the neuropathy is inflammatory based is a good idea...as it will also tell you whether it helped to reduce the inflammation, which can allow the nerves to heal. It's what you do with this information afterward that can be hard to decide.

I too have experienced the negative side-effects of steroids...and now have Cushing's disorder from taking too much. i can also NEVER stop taking one of my corticodsteroid/mineralocorticoid BP meds (steroids based) because now my body no longer makes this on it's own. Just one of the many gifts of steroids.

Steroids are really scary to deal with in the long term...which is one thing the doctors don't think too much about...their goal being to help you in the 'now'. Just be careful which ever way you go. If you try steroids, make sure it's a short course...just long enough to learn whether it is inflammatory based and whether the steroids helped...then stop and re-group to decide how best to approach along with considering all the other options out there (like IVIG). Make informed/educated decisions.

I have IVIG monthly for Immune Deficiency. Our Immune System protects us from pathogens, and mine is deficient. So I got lots of infections.

Now I don't get infections.

It hasn't affected my neuropathy at all. But it isn't at a dosage that is designed for neuropathy. And I understand that only a demyelinating sort of neuropathy can be helped with high doses of IVIG.

I do have demyelinating neuropathy...but it is profound, and I don't think the damage can be undone.

Anyway, that isn't on the table.

But the treatment is entirely safe. Only some people have difficult reactions to the process of infusion. I have NO problem with the infusion, but my own Immune System doesn't WANT the added IgG, so I have to take Medrol to stop the side effect when my Immune system kicks up a fuss over the 'guest IgG.'

It all works out and I go once a month and can tell anyone about the procedure who wants more information.

Hugs, ElaineD