Finally started IVIG

[Gingergrrl]

Dr Chheda doesnt think that I have any active infections at all, she thinks that I have Autoimmune Autonimic Ganglionopathy and MCAS (Even though I dont get rashes or anything)

Have they ruled out Myasthenia Gravis and LEMS?

So maybe this is why I'm feeling so much worse on a very low dose (5g) vs improving from a high dose? What do you think @Gingergrrl @Learner1 @Jesse2233 (I know Jesse hasn't been on for a while, but maybe I'll get lucky and he sees this)

I honestly don't know and I have never done low dose IVIG or SCIG. My understanding is that low dose is for immune deficiency and high dose is for autoimmunity (what I did for two years). But that it works as an immune modulator so depending on the dose it can be immuno-stimulatory or immuno-suppressant (but ideally normalizing your immune system from either direction). That is my non-scientific explanation!

 

[crypt0cu1t]

Have they ruled out Myasthenia Gravis and LEMS?



I honestly don't know and I have never done low dose IVIG or SCIG. My understanding is that low dose is for immune deficiency and high dose is for autoimmunity (what I did for two years). But that it works as an immune modulator so depending on the dose it can be immuno-stimulatory or immuno-suppressant (but ideally normalizing your immune system from either direction). That is my non-scientific explanation!

We have ruled out MG, but not LEMS. I think my symptoms almost perfectly match up with LEMS & Autoimmune Encephalitis. I still think that I have an unidentified antibody causing encephalitis.

This is definitely immune mediated, because I notice that I feel better and almost normal the next day after taking prednisone. (Still don't know why low dose IVIG caused me to go into this flare when High Dose put me in remission, but it reminds me of what @SK2018 experienced with IVIG)

Dr. Chheda has recommended rituxan if I cant handle the IVIG anymore, so before I go on with that I am going to see Dr. Schofield in Colorado to see if there are any other recommendations and if she has any experience with other immune suppressants (Imuran, Cellcept).

 

[Gingergrrl]

We have ruled out MG, but not LEMS. I think my symptoms almost perfectly match up with LEMS & Autoimmune Encephalitis. I still think that I have an unidentified antibody causing encephalitis.

In my case, we easily ruled out MG but we also could not rule out LEMS b/c I had the calcium channel (LEMS) autoantibody and many of the symptoms (although I was not a perfect match but LEMS seems to present in many different ways in different people). And in my case, I don't have any type of encephalitis.

I know I have asked you this a million times but have you been tested for the calcium channel (LEMS) autoantibodies (both the P/Q and N-type) and for anti-NMDA Abs?

(Still don't know why low dose IVIG caused me to go into this flare when High Dose put me in remission, but it reminds me of what @SK2018 experienced with IVIG)

It makes sense that high-dose IVIG put you into remission if your issues are auto-immune (vs. low dose IVIG would not be a high enough dose to affect your symptoms). But why low dose put you into a flare, I don't understand (b/c I just don't know enough about it).

And re: your PM, I am happy to help with what you asked me, I am just really behind with PM's as usual and got my Rituximab infusion on Fri and just starting to catch up with things today.

Dr. Chheda has recommended rituxan if I cant handle the IVIG anymore, so before I go on with that I am going to see Dr. Schofield in Colorado to see if there are any other recommendations and if she has any experience with other immune suppressants (Imuran, Cellcept).

That is amazing that you are going to see Dr. Schofield in Colorado! I think I told you that I e-mailed her (at least a year ago, maybe more?) and she replied and was very kind and helpful. She had a 2+ year waiting list at that point but she told me that she felt I was on the right track with my treatment plan and the only thing she might do differently (at that time) was to increase my IVIG dose even higher (to a 4-day cycle vs. a 3-day cycle) but I was already doing the highest dose that I could humanly tolerate so it was not possible.

Imuran and Cellcept scare me (b/c they can really wipe out your entire immune system vs. something like Rituximab that only targets the B cells and leaves everything else intact). However, if I were still in the physical state that I was in back in 2015 & 2016, and IVIG & Rituximab had not worked for me, I definitely would have tried anything (that logically had a chance) to help alleviate my suffering at that time. When do you go to Colorado?!!

 

[crypt0cu1t]

I actually have AChR Binding Antibodies which are usually correlated with MG, but can also be correlated with LEMS. I also have Salivary Protein 1 antibodies that correlate with Sjogrens and a high ESR/CRP but No NMDA antibodies as I once thought I did. I misunderstood the results from Stanford.

I don't know why low dose made me flare either but I havent been the same ever since that low dose. I've been contacting multiple doctors (Jaller, Frid. and I'm waiting on Schofield advice) and it seems like IVIG does exacerbate certain autoimmune disease like PANS, and Autoimmune Encephalitis through immune activation rather than suppressing like high dose does. Dr. Chheda doesnt think this is the case and believes my mast cells are flaring up, but I domt feel like that's all there is to it.

No worries about the PM, just whenever you get a chance to reply!

 

[Gingergrrl]

I actually have AChR Binding Antibodies which are usually correlated with MG, but can also be correlated with LEMS.

I don't think I realized that AChR antibodies can also be correlated with LEMS. If those are part of the Mayo Panel for MG and/or MuSK then I was negative for them.

I don't know why low dose made me flare either but I havent been the same ever since that low dose. I've been contacting multiple doctors (Jaller, Frid. and I'm waiting on Schofield advice) and it seems like IVIG does exacerbate certain autoimmune disease like PANS, and Autoimmune Encephalitis through immune activation rather than suppressing like high dose does.

I'm not familiar with the first two doctors that you mentioned but that is interesting that low dose IVIG can exacerbate certain autoimmune conditions. I guess that makes sense if low dose is meant to stimulate the immune system in someone who is immune deficient. Is that the reason why?

Dr. Chheda doesnt think this is the case and believes my mast cells are flaring up, but I domt feel like that's all there is to it.

For me (in the past), an immune reaction from IVIG was completely different than a mast cell reaction or histamine surge (whether minor or anaphylaxis). I have not had any IVIG since July 2018 (almost one year) but the two types of reactions were distinctly different for me.

No worries about the PM, just whenever you get a chance to reply!

 

[crypt0cu1t]

I don't think I realized that AChR antibodies can also be correlated with LEMS. If those are part of the Mayo Panel for MG and/or MuSK then I was negative for them.



I'm not familiar with the first two doctors that you mentioned but that is interesting that low dose IVIG can exacerbate certain autoimmune conditions. I guess that makes sense if low dose is meant to stimulate the immune system in someone who is immune deficient. Is that the reason why?



For me (in the past), an immune reaction from IVIG was completely different than a mast cell reaction or histamine surge (whether minor or anaphylaxis). I have not had any IVIG since July 2018 (almost one year) but the two types of reactions were distinctly different for me.

Yup, looking it up, it seems that AChR Binding antibodies are associated with Thymoma, Small Cell Lung Cancer, MG, LEMS or Autoimmune hepatitis, but considering I dont have MG or any tumors (that I know of) I'd say AAG, LEMS and AE is my best description.

Yeah the reaction I'm feeling is definitely not a histamine/MCAS reaction, it's a general worsening if my already existing AI symptoms. (Lethargy, visual issues, dysautonomia, headaches, hallucinations, muscle pain, neuropathy)

 

[Gingergrrl]

Yup, looking it up, it seems that AChR Binding antibodies are associated with Thymoma, Small Cell Lung Cancer, MG, LEMS or Autoimmune hepatitis, but considering I dont have MG or any tumors (that I know of)

I've Googled it now, too, and did not realize that AChR autoantibodies are also associated with LEMS and/or SCLC (Small Cell Lung Cancer). I had thought it was always associated with MG (and often Thymoma is part of MG). I was negative for this autoantibody but am now curious in your case if you have had a lung cat scan to check for small cell lung cancer?

I'd say AAG, LEMS and AE is my best description.

My doctor considered AAG as part of my differential diagnosis and a lot of these autoimmune dysautonomia's have a great deal of overlapping symptoms and autoantibodies. He ultimately felt that LEMS was the closest match to what I experienced except that I had much worse lung/breathing weakness and I did not have the classic leg weakness. (I never had any of the symptoms of AE).

Yeah the reaction I'm feeling is definitely not a histamine/MCAS reaction, it's a general worsening if my already existing AI symptoms. (Lethargy, visual issues, dysautonomia, headaches, hallucinations, muscle pain, neuropathy)

I agree what you are describing is not a histamine or allergic reaction. I am still confused, do you have muscle weakness or only muscle pain?

Just got some Good news that Dr. Chheda may be able to help me get plasmapheresis in San Diego! So I'll keep you updated on if this happens!

Wow, that is amazing and please keep me updated and I am curious where she would be referring you to in San Diego? You are still in NorCal, right? That seems far to travel unless it is the only place that she thinks would be able to do what is needed in your case?

 

[Jenc]

@Gingergrrl and @crypt0cu1t - did either of you see Dr Frid?

 

[Gingergrrl]

@Gingergrrl and @crypt0cu1t - did either of you see Dr Frid?

Who is Dr. Frid? I have not seen him (or her).

 

[crypt0cu1t]

@Gingergrrl and @crypt0cu1t - did either of you see Dr Frid?

I was going to see her, but she's extremely expensive.

 

[Jenc]

I was going to see her, but she's extremely expensive.

thanks - I am not sure if she's the one for me either

 

[Jenc]

Who is Dr. Frid? I have not seen him (or her).

Crypt0cu1t had mentioned dr frid - I got things mixed up when I tagged you!