Hi Nichole. I got dx with cvid in December. I wouldn't describe myself as a sick person prior to dx. I had mostly sinus infections. Maybe 4 Year? I had bronchitis some as a child and frequent tonsillitis. I was told if I had it more more time tonsils would come out...of course never got it again. I had a septoplasty and turbinate resection. About 5 years ago and had reduced number of infections. My main symtoms was fatigue. Oh how I rationalized my fatigue. I was so tired all the dang time. In turn I felt really depressed. But I thought I was depressed so I was tried lol.

I have been so fatigued I have almost passed out but I still wrote it off. I'm also a nurse so that explains a lot of my denial. I had been seeing my allergist who is also an Immuno. Went to see them because sinus infection wasn't clearing up and that is when they decided to work me up. Glad you are seeing one soon.

My ANA titer is elevated at 320 total complement is elevated, immunoglobulin a is non existent, g is low.

From talking to other people with this an Immuno it seems like most often people with autoimmune dx first then Immuno dx. There is also a lot of depression, fatigue and autoimmune associated pain. Have heard some about the enlarged lymph nodes.

Good resources for info: primaryimmune.org ( can get free resources mailed to you) IG living (free magazine), Just Like Me Magazine, my IG source (free info from Baxalta), Jeffrey Model, Foundation www.info4pi.com, idfcommonground.org,

Hi Nichole, I have lymph node problems too, only mine show reactive hyperplasia for no known reason. They looked for lymphoma and didn't find it. I see the oncologist again in a couple of weeks but I don't think they'll do any more biopsies or anything. I also have ITP and low IgG and IgA and some other deficiency with lymphocytes. Anyway, good luck with your immunology appointment.

Thanks guys. I saw the immunologist and we did the pre labs for the vaccine challenge and I got the pneumovax today. I'll go back for follow up labs in 5 weeks and see her back in 6 weeks. I will also likely get a high resolution ct of my chest to follow up my lung findings. She also checked the subclass IgG levels and again repeated my immunoglobulins which A was 9 and M was undetectable, IgG was pending. This will be the third time I've had them done. Funny how everyone wants their own labs.

The immunologist I saw seems bright but I am unsure how comfortable she is with immunodeficiency. I think she sees more asthma and allergy. She said something that clued me to this. She said she couldn't explain the lymphadenopathy. But from my research lymphadenopathy and more specifically the actual features seen on my biopsy are seen in CVID. I've read that on Uptodate and several other research articles. So I don't know...I left not feeling overly confident. But she seems to be familiar enough with the diagnostic approach.

She made a comment I didn't understand, maybe you all can help. She said if I respond to the pneumonia vaccine then I wouldn't need to see her anymore and that it means I have a working immune system even tho the levels are low and wouldn't need any treatment. I get you need to pass through the loops to qualify for treatment but I wouldn't go as far as to say if I respond to this one vaccine then I don't have a problem. I still have something going on in my lungs and lymph nodes that worry me. Albeit it may have been caught early but I want to keep it that way now in hopes of slowing any progression of lung disease specifically. So I'm not sure how she can write me off if I have a good vaccine response....

And Jen, I 100% with the fatigue thing. I was in graduate school for 3 years and then have been an NP for a year and a half. I also worked full time during school, got pregnant with our third during school so I had the excuses. I had reasons to be fatigued and tired all the time. But in the past year it has been so much worse. Again I assumed it had to do with work. Everyday life. Everyone's tired and so you just don't say anything because you assume everyone feels the same. And I'm not a complainer. But I just feel like something has to give at some point. I have thought depression, even have thought about getting on meds, anything to help me feel better.

Are you on immunoglobulin replacement?

Primaryimmune.org has a list of Drs who are experienced in treating cvid. I would go there and request Drs in your area. Just because you don't have cvid (possibly) doesn't mean you don't have something wrong with your immune system, what an idiot. I have met multiple people who have my same Immuno and he takes scare of many cvid patients. I wouldn't be comfortable with her. Your igg was low, iga is low and igm undetectable and that is normal? There are other immune diseases besides cvid. Sounds like she doesn't really know much about diagnosing other immune diseases.

Does she treat anyone with cvid? Will she know how to go about getting it approved through insurance? It would make me feel better to know if she has experience in this because it is quite a process.

I tried two different antidepressants previously that didn't help. Lost weight with one because I couldn't eat, which was nice to lose weight but not in that way! Other one couldn't sleep. I'm taking a different antidepressant now because I have headaches that we are trying to get under control. But I started a new Med that's working so I will wean off antidepressant.

I started in IVIG and had lots of headaches but not sure if because of IVIG I just get lots of headaches like 3-4 out of 7 days. Switched to sq for ease of infusions. I like it ok, definetely am feeling better overall. Felt tired last few weeks so we upped my dose and I think my immune system was fighting off some germs from my toddler.

I would finish the work up process but line up an appt with another Immuno for a second opinion. Go to primary immune.org click menu then resources then locate a physican

Hi NicholeU. I wanted to let you know you are not alone and I am empathize a great deal with your story, as mine has many similarities. I was quite healthy growing up - other than recurrent tonisillitis (had them removed at 17), and lots of weird GI issues that no one could ever figure out. However I did well in school and was a competitive athlete, including college - and I continue to be active as a runner, skiier, mountain biker, etc. I also work in healthcare (I'm a nurse too). My presenting symptoms that ead to my diagnosis were not infections either. I presented with joint pain and debilitating fatigue. My PCP insisted I see a rhemuatologist, who ran my immunglobulns. They all came back so low (IgG in 61, IgA undetectable, IgM undetectable) they thought it was a lab mistake. She repeated them and the IgG was even lower so she decided that those values were real and sent me to an immunologist. He repreated everything and my IgG was even lower by the time he ran it. He also performed that vaccine challenge and I had not mounted a response to the pneumovax or the tetanus vaccines. None whatsoever. He could not believe I wasn't much sicker with such profoundly low levels...I have since learned that there is a small subgroup of imunnodeficient patients who may present with few infections, but with many other problems that are autoimmune and/or lymphoprliferative in nature (such as granulomatous disease, ITP, autoimmune hemolytic anemia, enteropathy, and lupus-like or rheumatoid - like symptoms). I also have GLILD (granulomas in my lungs and liver) along with an enlarged spleen and lymph nodes.

They say that the autoimmune and lymphoproliferative problems can occur because when you are missing parts of your immune system, the whole immune system an become dysregulated. In addition to fighting infection, the immune system is supposed to be able to distinguish "self" (our own bodies) from "other" (invaders like bacteria and viruses). When the immune system is dysregulated, infections are not the only possible problem; the ability to distinguish self from other can also be dysfunctional. Once I understood that, it made perfect sense to me.

I would like to echo what JennBeth said about finding an immunologist who treats a larger volume of immunodeficient patients, even if you have to travel to get to one. It is extremely important. Many allergy docs have no idea how to manage patients with immunodeficiencies as they are quite rare.

The immunoglbulin labs measure the QUANTITY of the different immunoglobulins, and the vaccine challenge measures the FUNCTION of any immunoglobulins you do have. Some people may have low quantities of the cells, but the cells they do have actually function well and will protect them from infections. Other people may have higher numbers of cells but they may not function at all. People like me have very low number of cells and they do not functional at all.
Also, patient like us will often test negative for most autoimmune bloodwork ups because they are almost all based on measuring various antibodies - which immundeficient patients do not make. It takes a very experienced group of doctors to manage patients like us.

The granulamotous diseases that we can develop are often mistaken for sarcoid like diseases but they are NOT the same and must be managed differently than sarcoid would be managed. It is important because granulomatous disease can cause organ damage if not managed properly. For example, GLILD is a rare granulomatous lung disease that immunodeficient patients can develop that can lead to lead to pulmonary fibrosis. The good news is an experienced immunologist will know how to evaluate and treat this.

I am on IgG replacement therapy. It has helped with my joint pain and fatigue a fair amount, though it didn't resolve them completely. Once I started to get treatment for the GLILD , I felt an improvement in my energy levels. I travel out of state to see a specialist for the GLILD, and I have a local immunologist who collaborates with him to manage my care. It is a bit of a pain to have my main doctor so far away at times, but honestly it is the best decision I have ever made - to go see someone who really understands my disease(s).

I think it takes many patients some time to find the right providers for their care because these conditions are rare. Many of us have to travel to get the best care. I had to do a lot of research, get more than one opinion, and really advocate for myself in the beginning to get what I needed. It was scary, frustrating, and difficult at times but it really paid off to find doctors I felt comfortable with. I did a lot of my own research on primaryimmune.org and also read a lot of journal articles from peer reveiwed journals to help educate myself. It was exhausting at first, but really paid off in the long run.

I am sorry for the lengthy post but I wanted to try and share what would have been helpful to me when I was at the beginning of my journey with all of this. Hang in there and please know you have friends here to encourage and support you along the way.

Wishing you all the best!

Skiiergirl---very insightful post. Thank you. Definitely helps explain things to me where I can understand.

A couple of questions if you don't mind. What treatment are you on for the ILD? How did your ILD present? I am going to have a high res ct ordered when I go back to follow up and am concerned about some lung disease. I have normal PFTs and normal CXR but CT with contrast showed ground glass opacities and nodules. I actually work in pulmonary so I know overall these findings don't mean much on their own but I am wonder if, with all the immunodeficiency, if it could mean the beginning of some sort of lung disease. I am thinking I'd probably actually need an open lung biopsy to confirm.

Of course I don't want to be on any unnecessary meds but at the same time I want to be proactive and prevent complication such as fibrosis.

Another question-- does the Ig help to manage the other issues as well like the ILD or no? And if not, is it necessary to treat with Ig if not having infections. I am also the type of person who if I don't need the Ig I know there are people out there that need it and we rely on donors for the supply. So I am wondering how that all has been explained to you all.

Hi there and welcome. So, lots going on.

Immuno - as far as her dismissing you if your pneumo vax (did she also give you tdap? I had both but only failed 1). That will only rule out an antibody deficiency if you pass the pneumo vax. Could be other things as others have stated. I would perhaps consider seeing a doc who specializes in PI (the Immune Deficiency Foundation can provide a list - you may have to travel. I drive 5 hours one way to see my guy).

Autoimmune is tricky - IF you have an antibody deficiency than ANA and antibody based tests are unreliable in us. We may not mount enough of our own antibodies to throw a positive result in many tests.

I have GLILD as well. VERY different from regular ILD which is treated with long term steroids. We fail on steroids. GLILD happens in only about 20% of people with CVID, so were super rare lol. MOST people with GLILD present with SOB and impaired PFT's, fatigue. I do not get SOB. To me the logical process would be to get the CVID diagnosis and get treatment set up because being on optimal IGG replacement helps with the infections which helps with GLILD. Hope that makes sense.

Also, lymph nodes, I have enlarged all over, groin, abdomen, you name it. I've had a couple biopsied and they are not cancerous. Its common with CVID from what I've read and heard from others.

Some people with CVID are never sick. They just have GI issues, believe it or not. I actually was NOT sick my whole life and didn't start getting sick until I had my daughter, that triggered it for me.

Thanks Kelli. I was only given pneumovax not tdap.

How is you GLILD treated? I am hoping I do not have this. I don't get frequent infections so I'm hoping the high res ct with help clarify the nodules and GGO in the lungs. Did you have to have an open lung biopsy to confirm your diagnosis.

I live near a pretty large university hospital---Emory in Atlanta-- so I'm hoping if I need do get someone with more experience they might be able to help. They actually have a group dedicated to ILDs--not sure how much GLILD they treat but would be convenient if they did. It's about an hour and a half to them so not too bad overall.

The lymph nodes bother me BC I hate having to keep repeating CTs to monitor. I'm due in July for the repeat. My biopsy showed granulomatous disease which is why rheum thought sarcoid but from what I've read through my research is that CVID can have a sarcoid like appearance on biopsy so I don't really know how to go about knowing whether it is sarcoid or an effect from CVID or if it needs to be treated or not. I just need a diagnosis first I know but this 4-6 week wait for the vaccine challenge is killing me

So the docs who specialize in GLILD usually require an open lung biopsy for diagnosis because patients are often misdiagnosed without one. Also, the biopsy needs to be assessed by a pathologist who has experience with GLILD otherwise they will not know what they are looking at and will misdiagnose it - even at institutions with good reputations, even if they have an ILD dpartments. Many good pulmonologists/ILD departments have never diagnosed it or treated it because it is so rare. I think the key is to find out if whomever you choose to see has any experience diagnosing and treating GLILD. Many of us fly to see doctors who have been involved in writing papers about GLILD to make sure we go to someone who is actually familiar with it. There are only a few who do so unfortunately it ususally requires travel to see them. Many of these docs treat with a combination of imuran and rituxan, or other similar agents. I had the open lung biopsy to get diagnosed and am on the imuran/ritxan protocol. But we are all different, so I want to be clear that it is best to get the opinion from an experienced immunologist on all of this.

Hang in there, I know it is really difficult to wait around for all these test results! Personally that part of the process was the hardest for me. During my waiting period, I did a lot of research and tried to educate myself as much as possible on everything. It helped pass the time and helped me prepare questions so I could advocate for myself. But it is totally worth it in the long run to have the proper work-up and get a correct diagnosis, as CVID is not the only possibility; there are other types of primary immune deficiencies and it is essential to get the correct diagnosis. The IDF has a lot of resources online as well as hard copies they can send to you that really help explain a lot of this, I highly recommend checking out what they have available at pirmaryimmune.org.

Make sure to get hard copies of all of your test results, scans, etc. They are important for insurance records and in case you ever decide to get any second opinions on anything.

Hang in there!

Hi Nichole, I am new in this group too, in fact, this is my first time posting. I was reading your story and in a lot of ways, it is so much like mine. I have CVID with probable GLILD (I have never had an open lung biopsy, only a bronchoscopy, but my doctor thinks I have it).

I too, have never had problems with chronic infections,but have had problems with a chronic cough. I started a long process of trying to figure out what was wrong with me in spring of 2013 when I had a sudden appendicitis attack and the CT showed I had enlarged lymph nodes all over my abdomen and pelvis. The ED doc told me I had ovarian cancer, but during surgery they looked at my ovaries and they were fine. Then I started a year of going to an oncologist who thought I had lymphoma. I had a CT-guided biopsy of a retroperitoneal node and removal of 2 enlarged nodes under my arm that all came back showing only reactive cells, so thankfully no cancer. My oncologist was stumped. I think he did some research checking out differential diagnoses on me because he added on some labwork after seeing me and drew my immunoglobulins which turned out to be all low and he thought I might have an immune disorder. I also had lung nodules and ground glass opacities on lung CT, so he sent me to National Jewish hospital ( I only live about an hour away). Even National Jewish at first sent me down a sarcoidosis path, but finally got me to the right doctors. I am seeing an immunologist and an interstitial lung disease pulmonologist and got started on monthly IVIG infusions October 2014. These are going well and my baseline IgG is now in a low normal range. My fatigue is better, but not gone. I have pulmonary function tests twice a year which are okay for now, but I may need to go on oxygen at night. I have some abnormalities in my white blood count and platelets too, but they say these are stable. I guess most my issues are from autoimmune complications of CVID and not CVID itself. From your story, I would be surprised if you did not have CVID. I am a nurse too, by the way. :-)

Second everything skiiergirl said. There are only a few who treat and know a lot about GLILD. It's pretty important that you don't get stuck with a regular ILD person who wants to put you on long term steroids. And these docs do usually ask for an open lung biopsy. One absolutely requires and will not treat without it. Although I had one, I'm still not on treatment. I do ok day to day, work full time, my infections are well under control and my IGG remains high, which help the GLILD.

COKim - I wanted to add that you could still really be impacted by fatigue if your IGG isn't high enough. We all have a "magic" number we do well at and its different for all of us. I find I do best if my IGG is 1300 or higher. It really helped with the pain I have from the arthritis I got from the CVID.

Thanks everyone. I feel like it's easier to post here and have a conversation about this rather than with family or friends. They don't get it.

Still waiting on the vaccine challenge. I get my follow up blood work in 2 weeks then I will see the immunologist to get the results and see if we can get a diagnosis. Will also be getting the high res ct ordered as well as my follow up ct neck abdomen and pelvis to follow up the lymph nodes.

If the high res ct of my chest shows anything that could be indicative of the GLILD I will ask for a referral to someone who can diagnose it.