Primary immune deficiency.......anyone?

[heapsreal]

I'm beginning to think so. How many of us have had adequate immune testing to know whether or not we have some primary immune deficiency? Not many, all told.

Certainly we've seen low NK cell function (Klimas, Bond Uni), and I'm betting I'm not the only one with CD deficiency given how many of us have persistent viral infections, chronic bronchitis, or chronic sinusitis.

It's gonna piss me off if a diagnosable T-cell (CD8) deficiency, indicated by chronic bronchitis, multiple bouts of pneumonia, persistent ebv symptoms, and catching everything under the sun, was missed by my GPs all these years. I mean, I've been telling my GPs for decades that my immune system doesn't seem to be right and they just blew me off. I mean, jeez, what are we paying them so frickin' much money for?

Can you tell I'm in a bad mood tonight? Baby didn't get her nap today.

same, ebv/cmv mono with sinusitis etc, doesnt seem alot of treatment out there from PID other then treating infections directly and immunoglobulins. Might be why they cant find 'ONE' cause of cfs/me.

cheers!!!

 

[IreneF]

IreneF

I am only familiar with CVID being treated with immunoglobulins. When I get a chance, I am going to look into Rituxan. How long have you been on treatment and are you feeling better?

As far as what you posted making sense, I find the whole darn immune system very confusing. It seems the more I read the more confused I get. Sorry I could not be of any help there.

June

CVID usually involves an antibody (immunoglobulin) deficiency. I wonder if my doc used it as a diagnosis because being diagnosed w/ CFS is the kiss of death, treatment-wise, and CVID comes the closest to describing my condition.

Dr. Kogelnik is the only physician in the US treating the-condition-formerly-known-as-CFS with Rituxan, altho it is approved (I think) for certain autoimmune disorders.

I had my first infusion at the end of March, one more at the beginning of April, and I am scheduled for a third around Memorial Day. I feel about the same as I did before--not good, but certainly no worse than before. I got a fairly mild reaction to the first treatment with fever, pain, and more fatigue than usual. I am not expecting to see any improvement for several months.

Because Rituxan wipes out the antibody-producing cells it's probably not a good idea for people who are already deficient in that respect. It might possibly be beneficial if you have symptoms caused by excess cytokine signaling from B cells but I don't know if anyone has explored that aspect among patients with classic primary immune deficiencies.

Yes, the immune system is one of the most complex aspects of biology. Tough to follow when one's got brain fog.

 

[IreneF]

Most PIDs are probably genetic, so there isn't any any way to treat them other than with antibiotics and Igs.

 

[taniaaust1]

Most PIDs are probably genetic, so there isn't any any way to treat them other than with antibiotics and Igs.

Still would be great to know about if one has this issue.

 

[DaiWelsh]

I asked my GP (UK) about CVIDS recently and I am getting the IGg blood test done next week, however when I suggested to my GP that this might get me out of the wilderness of no-treatment CFS he said in his experience people with CVIDS went to the immunologist who said "interesting diagnosis, nothing we can do about it". Anecdotal but not encouraging, is this just UK again?

 

[IntuneJune]

Rituxan

IreneF

Yes, you are quite right,CFS as a diagnosis is the kiss of death resulting in treatments that are not of much help.
There are so many primary immune deficiencies and over the years, I have gone from a subclass deficienty to common variable. The treatment however was the same.

There are some patients who do have CVID and elect not to treat with infusions. The patient's histories seem similar however, they have not had repeated very serious infections...so they are gambling and hoping they don't get struck down. I did not get diagnosed until age 60, and my past history reads very similar to those who got diagnosed at a late age, frequent severe infections, pneumonias, bronchitis, sinus infections with resultant pathology of the lungs and sinuses because of it and other autoimmune problems.

If you did have a dx of CVID, treatment with immunoglobulin should have been offered you. So maybe you are correct, it was the "closest" and got you a trial of Rituxan.

I hope it helps you. June

QUOTE=IreneF;256890]CVID usually involves an antibody (immunoglobulin) deficiency. I wonder if my doc used it as a diagnosis because being diagnosed w/ CFS is the kiss of death, treatment-wise, and CVID comes the closest to describing my condition.

Dr. Kogelnik is the only physician in the US treating the-condition-formerly-known-as-CFS with Rituxan, altho it is approved (I think) for certain autoimmune disorders.

I had my first infusion at the end of March, one more at the beginning of April, and I am scheduled for a third around Memorial Day. I feel about the same as I did before--not good, but certainly no worse than before. I got a fairly mild reaction to the first treatment with fever, pain, and more fatigue than usual. I am not expecting to see any improvement for several months.

Because Rituxan wipes out the antibody-producing cells it's probably not a good idea for people who are already deficient in that respect. It might possibly be beneficial if you have symptoms caused by excess cytokine signaling from B cells but I don't know if anyone has explored that aspect among patients with classic primary immune deficiencies.

Yes, the immune system is one of the most complex aspects of biology. Tough to follow when one's got brain fog.[/QUOTE]

 

[IntuneJune]

DaiWelsh
Here in the US, when a patient receives a dx of CVID, they are treated with immunoglobuins......this prevents or drastically reduces serious infections. I do know of one person who refused treatment concerned about receiving a plasma product.

The subclasses should be included in your testing.

 

[IntuneJune]

IreneF

One of the most respected pediatric immunologist's, Dr Bonilla, spoke at an IDF meeting and was asked this question. Are immune deficiencies inherited? He replied there was no data to support this. I was sitting at a table with a Mom whose children had an ID, she has, and her father! Humph!!!! There was his data.

June

 

[IntuneJune]

Wally,

Thank you for the links. I have followed folks here (some time ago) who have had office visits with these physicians, but cannot remember their outcomes

I will visit their sites.

It might be that because of an immune deficiency, a virus was able to get a good hold and settled deep into our bodies...most folks get a virus and get better, then some of us don't. It would make sense that the ones who don't have an immune deficiency.

June

 

[IntuneJune]

not just autoimmune but also persistent viral infections.

cheers!!!

Ohhhhhh!!!!! I wonder if the viral proflie differs from the autoimmune?

 

[IreneF]

IreneF

One of the most respected pediatric immunologist's, Dr Bonilla, spoke at an IDF meeting and was asked this question. Are immune deficiencies inherited? He replied there was no data to support this. I was sitting at a table with a Mom whose children had an ID, she has, and her father! Humph!!!! There was his data.

June

He may have been correct, if no one had published a case report. Saying "no data" is just another way of saying "we don't know". It's not the same as IDs are never inherited.

This looks like someone's class notes, but if you don't follow medicalese you will find it less than an enjoyable read:
http://www.opt.uab.edu/class2011/2nd year/micro/transcripts/Immunodeficiency Transcript.doc

Irene

 

[heapsreal]

my immunoglobulin sub classes have always been high, mainly IgA.
In japan i have seen it written that they call cfs, low natural killer cell syndrome, i think this name describes many cfsers immune dysfunction better

 

[Stellar0]

Hi June. I started out with an EBV that was active for 3 years. I was dx with fibro in the middle of that infection. Almost like clock work at the end of the 3rd year of the ebv I contracted West Nile Menegitis that lasted exactly 1 year and last but not least I moved into a house that aspergillus penicillium in it. You got it.... it attacked me with a vengence. I had mycotoxicosis. Now after all this my doc decides she needs to check for CVID and voila! I was put on Hizentra SCIG in May 2011. My most recent sick badge is for my new dx of Hoshimoto's. Please free to message me if you like some support and to trade notes.

 

[IntuneJune]

CVID and throid issues

Stellar, this is very common; many, many primary immune folks develop thyroid issues and other autoimmune problems.

This past weekend, I went to a regional conference for PIDD and thryoid diseases were discussed. Sigh, we get it "coming and going."

Does your immuno believe you could not fight the other issues because of a dysfunctional immune system, or did the other ongoing issues wear your immune system out?

June

 

[Ocean]

Many patients would qualify for a diagnosis of primary immunodeficiency if appropriate testing were done. "CFS" is a diagnosis made by excluding certain other illnesses, while PI is a diagnosis made on the basis of certain positive findings. It is better to get a PI diagnosis because you are much more likely to get treatments paid for.

The most widely recognized and serious form of PI, low IGG levels across the board, does not apply to most CFS patients and probably excludes the diagnosis.

But, more minor kinds of PI are very frequently found in those with a CFS diagnosis initially:
-IGG subclass deficiency, especially subclass 3 (B-cell dysfunction)
-Failure to respond to pneumococcal vaccine or other vaccines by producing antibodies (B-cell dysfunction)
-Mannose-binding lectin deficiency
-reduced T-cell proliferation in response to mitogen stimulation (T-cell dysfunction)
-gamma clonal T-cell gene rearrangement (T-cell dysfunction, pre-lymphoma)
-NK cell deficiency, or reduced NK cell function + multiple recurrent herpes virus infections (T-cell dysfunction)

Having a specific immune defect makes it much more likely that you can get insurance companies to pay for the more expensive treatments. They are especially sympathetic if you have B-cell disorders and your doctor is seeking treatment with IVIG or Rituxan.

So having low levels of one of the IGg subclasses wouldn't qualify one for a diagnosis, right?