Immune system

[Biddy47]

Hi everyone, I wonder if anyone can enlighten me. I was diagnosed 5 yrs ago with cutaneous lupus and sjogren's syndrome, I had blood tests and skin biopsy, I take Hydroxychloroquine. Because I have been having frequent infections I had a blood test for immune deficiency, they came back normal except for a mildly lymphocyte count which was 1.13. I'm confused, can anyone explain? Thanks.

[Paul_Howard Partner LUPUS UK]

Hi Biddy47 ,

Was there a range provided with the result? Did they indicate whether your lymphocyte count was high, low or normal?

[Biddy47 in reply to Paul_Howard]

Sorry Paul, I missed out a word. He said that it was mildly low at 1.13. and that that is consistent with a viral illness.

[Paul_Howard Partner LUPUS UK in reply to Biddy47]

OK. Have they suggested any action to address this? Are they making any changes to your treatment or doing any further tests?

[Biddy47 in reply to Paul_Howard]

No. changes or treatment. The blood test was taken by my chest consultant.....I also have bronchiectasis, and the reason for the test was that after any infection it was taking up to 8wks. to fully recover. The letter with the results that I received just said that my kidney function and full blood count are essentially normal apart from a mildly low lymphocyte count at 1.13, which is consistent with a viral illness. It also says that my immune function tests show normal immunoglobulins and no gross evidence of immune deficiency. If I have 2 autoimmune conditions, how can this be? I'm confused to say the least.

[123MrsG in reply to Biddy47]

Hi biddy, I also have a chest condition, similar to bronchiectasis, as well as SLE. It seems to me that an ‘immune deficiency’ and an ‘autoimmune condition’ are not the same thing. I think your chest consultant was probably checking to see if your immune system is underactive to see if that might be a factor in how long it is taking you to recover from infections. The tests used in that respect won’t be the same tests that measure autoimmune disease. I’m not sure how possible it is to have an autoimmune condition as well as an underactive immune system, but I’m sure your chest consultant had a good reason to run the tests. I know the question remains as to why it’s taking a long time to get over infections. In my own experience of having CF, sometimes it just takes a while to get over an illness and that’s just how it goes.

[Barnclown in reply to Biddy47]

Hello biddy: good questions! overlapping forms of immune dysfunction & connective tissue disorder happen a lot, and some of us even have primary autoimmunity simultaneous with primary immunodeficiency and primary connective tissue disorder.

Eg i am 65 and my 3 overlapping primaries are Hypermobile Ehlers Danlos + infant inset systemic lupus + v early onset antibody deficienct disease (hypogammaglobulinaemia + lymphopenia). Of course i also am diagnosed with a long list of secondary conditions

So, my lead clinics are rheumatology & immunology, but i also attend a long list of other clinics regularly: gastroenterology, ophthalmology, ENT etc etc. All my issues have to involve multidiscipline liaison. Cases like mine are unusual, but i know others with mixes like mine, thanks to several wonderful internet forums & support groups including Lupus UK

Over my 7 years here, i’ve discussed the manifestations, diagnosis & implications of my unusual diagnostic mix quite a lot on forum...so i won’t ramble on now. But here is a link to a great support group website that explains the spectrum of immunodeficiency diseases:

piduk.org

Feel free to private message me if you would like to chat about this

🍀🍀🍀🍀 Coco

[Pussbella]

Dear Biddy,

I might be able to help as I was in a similar situation to you. Lots of infections which took ages to clear despite antibiotics etc.

I, too, had the immugoblin tests which came back relatively normal but as the Ig G one was quite low my immunologist did further test looking at specific antibody levels. It turned out I had extremely low antibody levels for pneumococcal and Hib. I had two separate vaccinations a few weeks apart to try to provoke an antibody response. It was successful for Hib but not for pneumococcal.

This meant a diagnosis of specific antibody deficiency. You might want to Google it because it could be relevant for you. The link Barnclown has provided is excellent.

In my case my symptoms are mild but for some this is not the case and specific antibody deficiency (sometimes known as Spad or Sad) can lead to CVID (chronic variable immune deficiency).

I now have developed autoimmunity as well with a very high Ana and a diagnosis or Uctd/? Sjogren's. Like you I wondered how one can have an overactive immune system along with a deficient one. My immunologist described the it in this way. If one part of the immune system is not working another part might become even more active as a response to this. I imagine it like armies in my body I have lost one division and another division has gone a bit wild to compensate!

Recently I have been tested again to see what the pneumococcal antibodies are doing - if anything - so I am being monitored which is useful. It also means there is no argument when I might need antibiotics for longer than usual.

Along with the antibody deficiency I had an extremely low Mbl (mannose binding lectin) level. This, too, can predispose you to infection and make it difficult to clear infections. My rheumatologist commented on the Mbl level when he saw it. However, the immunologist said because the Mbl level is a part of the immune system which is innate - there is nothing you can do about a low Mbl level But again I find it useful to know. Again this might be useful to Google.

Perhaps you could have some further tests to look for specific antibody deficiency and Mbl levels and your complement levels or maybe all this has been done already?

Very much hope this not very scientific explanation helps! But overall you can have a primary immune deficiency and autoimmunity.

Bw

Pb.

[Biddy47 in reply to Pussbella]

Thanks Paul for replying to my post and many thanks to , Mrs. G, barnclown and pussbella. Your posts have been a great help in helping me to understand all this. I too had low antibodies to HIB and received the vaccine with brought them to normal levels. To be honest my rheumatologist is not much help at explaining things to me. He just said that my body is not very good at coping with infections! Well I know that. I'm more than capable of understanding things when explained to me. He specialises in another field of rheumatology so not sure how informed he is about lupus/sjogren's, although being a consultant rheumatologist he should be. Thanks again to you all.

[Barnclown in reply to Biddy47]

You’re v welcome...and thanks for this great discussion. Your rheumy is either not well informed or being rather patronising...this is a complicated subject, but basic info is graspable....as i know from my immunodeficiency forums. My rheumatologist does understand immunodeficiency, but i tend to discuss it with my immunologist...if i could refer you to immunology i would, pronto!

Immunology took several years to more fully understand my immunodeficiency including proving it’s not due to my immunosuppression meds. It’s v good you’ve produced antibodies in response to these vaccine challenges..i’ve proven incapable of creating and antibodies in response to both types of pneumonia vacc & to haemophilus vacc, and i had virtually no antibodies anyway, so now i’m ultra vulnerable...due to this i’ve been on daily antibiotics for years and am to start immunoglobulin G replacement therapy soon (sub cut igG) cause last spring pneumonia broke through the antibiotics. Fortunately my univ hospital team + the PID UK forum are a HUGE help and i feel relatively ok about all this immunodeficiency stuff.

Take care & good luck😘🍀😘🍀😘

[Biddy47 in reply to Barnclown]

Gosh that's a lot to contend with, it puts my problem in the shade, you seem to have a good team looking after you so that's good. I wonder if perhaps the infections, which include UTI's actually cause a bit of a flare and that's what makes me feel so unwell for so long. When I get through these symptoms I feel really well, apart from some fatigue which I can cope with. Thank you all again for taking the time to explain things, it really has helped me understand a bit more.

[Barnclown in reply to Biddy47]

You’re totally welcome! You’re helping me too! Putting this into words helps me A LOT...the brain fog etc mean i need to keep refreshing my memory re what this is about

Yes indeed: my autoimmunity always flares during infections...even while these are slowly clearing up thanks to being treated with extra antibiotis. I always check myself all over when AID is flaring just to make sure i’m not missing an infection starting up. Immunology says i have persistent pockets of chronic bacteria driven sepsis due to my immunodeficiency going without adequate treatment for 50+ years. (In jawbone, respiratory system, stomach, urinary tract etc). These are all responding pos to immunology’s treatment plan even though i’m needing all rheumatology’s combined therapy immunosuppression meds (hydroxy + pred + myco). Which seems contradictory but REALLY works well for me!

[Pussbella in reply to Biddy47]

Glad the reply helped.

I have one more thought. You said you had low Hib antibodies which then responded to vaccination. There are a group of people who initially respond to vaccination as you did but the response is shortlived and lasts only a few months after which the antibodies return to low levels. Maybe this should be checked to see if it has happened in your case?

Specific antibody deficiencies are linked to chest, sinus and ear infections. Like you, however, I get lots of Utis and like you I have noticed autoimmune flares seem to affect the severity of infections and vice versa.

Again, hope this helps.

Bw

Pb

[Biddy47 in reply to Pussbella]

Thanks Pussbella, that's interesting but I have to say that since I got the vaccination, which was 6 years ago, I haven't had an HI infection which to me is amazing because that was the bug that plagued me for years. I've had many other weird and wonderful bugs but thankfully so far not this one, so it seems to have worked for me.

[Barnclown in reply to Pussbella]

Fascinating to read your reply pussbella: am so glad you’re here!

Isn’t it good when our rheumatologist & immunologist work together this way...having this attention makes me feel vvvvv lucky ...but indid have a dreadful time for 50+ years before rheumatology realised my immunodeficiency was there all along and explained my seronegativity as well as all the complex persistence pattern recurring multisystem infections + increasing antibiotic resistance etc etc etc. I explain above in reply to biddy that i have neither pneumonia nor haemophilus antibodies & fail to make any in response to vacc challenges so am on antibiotics long term...and soon to stary IgG replacement therapy

😘🍀😘🍀😘🍀

[Pussbella in reply to Barnclown]

Thsnk you!

I have learnt a great deal from your posts. You have so much with which to contend.

Best wishes

Pb

[Ericanemec]

I would change meds. You are probably getting sick with infections because your immune system is TOO LOW!

Whether you believe it or not,everyone needs an immune system, and it sounds like yours is being suppressed just way too much. I would ask to switch to something that doesn't exhaust your immune system into being almost non existant.

I have Lupus,RA,Fibro and many others including sub acute cutaneous lupus (which is just a skin rash due to lupus).

There are different types of lupus and I think you deserve better medical treatment. You shouldn't be getting frequent infections due to the fact your immune system is being slaughtered by meds.

[Biddy47]

Thanks Ericanemec, the only medication that I take for the condition is Hydroxychloroquine which I understood was one of the milder meds. Which meds if any do you take?

[Ericanemec in reply to Biddy47]

It's not mild at all.

[Ericanemec]

I take prednisone for flares and that is all.

[Ericanemec]

There is a reason you have Lupus. My reason was low IgG immunoglobulin protein in my DNA. I did orencia infusion treatments for 1 year to replace the protien. I have not had to take anything daily for years because it fixed me right up with the exception of an occasional flare. Tell the doctor you want to find out WHY you have lupus to begin with. Most doctors just mask it by killing your immune system, which isnt even logical. Fix the problem at its source and you will help fix the Lupus.

[Biddy47]

Thanks Ericanemec for your comments. I do find the whole issue of the immune system very confusing.