Selective IgA Deficiency

    • Anonymous
      February 2, 2009 at 7:59 pm

      Hi All,

      I had MFS/GBS in September ’07. At the time, my neurologist checked my IgA level. It turns out the level was very low (which is not good).

      “The IgA class of immunoglobulins has the specific function of protecting the body’s mucosal surfaces (eyes, mouth, throat, lungs, gastrointestinal, and genitourinary tract) from infection. While IgA is aided in this role to some degree by other classes of immunoglobulins, the lack or severe deficiency of IgA at these body sites makes one or more prone to recurrent infection, allergies, chronic diarrhea, or autoimmune diseases. In autoimmune diseases, the immune system mistakenly attacks the body’s own tissues.”

      http://npi.jmfworld.org/patienttopatient/index.cfm?section=patienttopatient&content=syndromes&area=2&CFID=3169571&CFTOKEN=28540

      My neurologist tested me for this to identify which treatment I should get. If the IgA number is low, it’s possible that your body may have created an antibody against IgA (another test, which has to go to the Mayo Clinic, and takes time to get back – so I’m guessing neurologists just look at the IgA levels), and if this is the case, then a person is at risk for anaphylactic shock if receiving whole blood products (e.g., IVIG).

      Since my IgA number was low, I got plasmapheresis instead of IVIG.

      I met with an immunologist after my whole MFS/GBS episode, and he tested me further to make sure my other antibodies (that perform other immune system functions) were OK, and to see if my body created the IgA antibody

      It turns out I have Selective IgA Deficiency [U]and [/U]the antibody against the IgA antibody. My other antibodies were normal.

      (By the way, they say that Selective IgA Deficiency is the most common immunodeficiency. One in 500 people have it and most never know they have it unless they experience something like this, as my immunologist told me. In some people there are little to no symptoms; on the extreme end, people are prone to sinus infections, etc.)

      [B]Long story short – I’ve been pondering if the Selective IgA could have been a possible cause for my MFS. My MFS was precipitated by the campy bacteria, and lack of IgA leaves your body at risk for gastro infections and autoimmune diseases. So I’m wondering if there’s a connection between the Selective IgA and my MFS.

      I’m also wondering if anyone else on this forum has been diagnosed with Selective IgA Deficiency.[/B]

      I know no one has the answers. (Including doctors – I asked my immunologist and he did not think there was a connection; however, has anyone ever studied this or identified a link between the two?) I’ve just been pondering this over the months, and wanted to see if anyone else has any insights.

      Thanks,

      Paula

    • February 2, 2009 at 8:44 pm

      This is very interesting. Next time my older son needs blood work, I am going to suggest this as he is plagued with chronic sinus infection. Thank you.
      Dawn Kevies mom

    • Anonymous
      February 3, 2009 at 12:03 am

      Paula,

      I read on one of your previous posts that your GBS was caused by the Campy bacteria. How did they determine that was the cause?

      Thanks,
      DonnaM

    • Anonymous
      February 3, 2009 at 1:54 pm

      DonnaM,

      They tested for it in the hospital…

    • February 3, 2009 at 3:40 pm

      While investigating this further, I also found that certain fungi are linked to selective IgA. My son has tinea versicolor. It will be hard waiting till April when we see this dermatologist. thanks again for the info.
      Dawn kevies mom

    • Anonymous
      February 3, 2009 at 5:38 pm

      Hi, I have a few comments on selective IgA deficiency. First, as you said, it is pretty common at 1 in 500 people. It is rare in peoples of Asian descent. Second, if you search literature for IgA deficiency and something, you get not only selective IgA deficiency but also other reasons for immunoglobulin deficiency including common variable immunodeficiency as well as many more. This confuses associations with other diseases because non-selective immunodeficiencies are associated with more diseases that selective deficiencies are. Also many people lower than normal in IgA are called IgA deficient when they really have rather a lot–just not the normal amount. The fact that you have anti-IgA antibodies argues that you are really very low. I work at a children’s hospital with children with cancer and immunodeficiency diseases. I have always heard that the risk of anaphylaxis to blood products (including immunoglobulin) infusion is 1 in 20,000 so being IgA deficient is not enough nor is it to have anti-IgA antibodies since most anti-IgA antibodies are of the IgG1 subtype and it is very rare for them to be of the IgE type. I saw a good paper on IgA deficiency “Clinical significance of immunoglobulin A deficiency” in Annuals of Clinical Biochemistry, 2007, vol 44, p131-139. It lists a lot of autoimmune diseases–17 of them associated with IgA deficiency–especially celiac disease, but GBS/CIDP are not on the list. Myastenia gravis was the only neuromuscular disease. It also states that having IgA deficiency and anti-IgA antibodies are not reasons to exclude the possibility of immunoglobulin infusion. Here we test IgA levels before the first immunoglobulin infusion for which an alternative might be available and also as a precaution. If you were to get immunoglobulin, you are a person that should have it had a place where anaphylaxis could be immediately treated and with special precautions. The point of this is that you could get it, it just should be considered carefully and you might want to get subcutaneous immunoglobulin instead of IV. the problem with subcutaneous immunoglobulin is that it is not as studied in GBS/CIDP. There are also products of immunoglobulin that are lower in IgA levels and you would likely want one of these so your anti-IgA antibodies formed fewer immune complexes. If plasmapheresis works well–great–but if it does not alternatives including possibly immunoglobulin given carefully could be considered.
      WithHope for a cure of these diseases

    • Anonymous
      February 3, 2009 at 8:53 pm

      Paula,

      The reason for my earlier question concerning how they determined it was Campi Bacteria, is because that is what I think caused my CIDP. I had a colonoscopy and the next week the muscle fatigue and the muscle weakness started. It was six months and two neurologists later before I was diagnosed with CIDP. To my knowledge there has never been a test for Campi bacteria. I also have abnormal liver counts. I have had an Ultra Sound and liver biopsy and nothing is found to cause the high ALT and AST. My liver has looked fine for both tests. The doctor who specializes in the liver lectures me about drinking. I counted up and I had seven glasses of wine in 2007, but was a bit worse in 2008 with 10 glasses of wine and one margaritta. He looks at me like he does not believe me. I keep asking him if I have or had an inflammation of the liver due to a bacteria, what would his medical advice be. He said the same.
      I do not know if it makes any difference at this point to know what caused the CIDP. I receive IVIG and have made progress although it seems to have leveled out which is quite disappointing. I guess my question to you now is if CIDP is caused by the Campi bacteria, is there anything different that should be done?
      Thanks again,
      Donnam

    • Anonymous
      February 4, 2009 at 7:03 pm

      WithHope – I’m not so much concerned with needing IVIG. I’m a year and a half post-MFS. I was just curious about links between Selective IgA Deficiency and GBS. I’ve seen the list of auto-immune diseases (usually they say they are linked to other immune system deficiencies), and I too have not seen GBS on that list. But there seems to be so little research done on GBS, that anything is possible. Thanks for the information you shared.

      DonnaM – I’m sorry I can’t answer your question about whether or not something different should be done for your CIDP if it was caused by the campy bacteria. I don’t have CIDP myself, and am only aware of what I’ve read through this foundation. But with GBS, I think that regardless of the cause, the treatment is the same. So it might be with CIDP. I hope you find the answers you’re looking for. There are many people on this forum who I’m sure can provide you with some insights.

      My MFS started with a gastrointestinal infection 2 weeks before my first MFS symptoms. The CDC says that up to 40% of patients with GBS have evidence of campy infection when the neurological symptoms begin. Do you remember anything like that at the time of your colonoscopy?

    • Anonymous
      February 4, 2009 at 9:57 pm

      Paula,

      No I had absolutely nothing other than the colonoscopy. I was told this was something I should do at my age and I finally broke down and had it done.

      I had an rather “crusty” nurse blurt out when I told her the only thing that had happened to me was a colonoscopy the week before — “He did not clean his equipment.” Of course I have no proof of that. But, I do feel my CIDP was the result of an infection. If the treatment is the same regardless of the cause, then I will just continue. I also feel my liver counts are the result of inflammation from an infection. But again if all require the same course of action regardless of the cause, then I am doing all that can be done. I just keep reading references to Campi Bacteria and wondered if something special should be done if that were the cause.

      Thank you for your information.

      DonnaM