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Celiac Disease and the Enteropathy Associated with Common Variable Immunodeficiency


Celiac.com 07/09/2010 - The enteropathy associated with common variable immunodeficiency (CVID) is the most common  symptomatic primary antibody deficient syndrome, with an estimated prevalence of one in one-hundred thousand to one in fifty thousand. However, the relationship between CVID and Enteropathy is still unclear.

CVID is characterized by decreased levels of of two or more serum immunoglobulin (Ig) isotypes and the presentation of reoccurring infections specifically in the respiratory tract. Gastrointestinal symptoms are widespread with CVID patients as exhibited in as many as 50% of patients presenting with chronic diarrhea.

A team of doctors evaluated the medical files of 50 CVID patients who exhibited gastrointestinal symptoms to determine the “clinical and hitopathological features of the enteropathy associated with CVID”. Fifteen patients were excluded from the study because they did not meet the recognized criteria for CVID. Data was collected from all patients and included, gender, age, symptoms, body mass index (BMI), as well as parasitological stool testing. Blood samples were taken from each test patient including hemogram, serum protein electrophoresis and measurements of serum folic acid, vitamin B12, iron, and calcium.

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The doctors found the mean age for initial CVID diagnosis to be 36.8 years. Four of the patients were discovered to have a family history of immunodeficiency. 40% of the patients that were tested were determined to have immunodeficiency as revealed by their digestive symptoms. Chronic diarrhea was observed as the most common gastrointestinal symptom with a rate of 92% of the patients studied.

Gluten-free diet was initiated by 12 patients with villous atrophy, but clinical improvements and partial villous healing only occurred in two patients. Interestingly,  the two patients presenting with celiac antibodies,  did not show an improvement of symptoms. All patients showed positive improvements from steroid therapy. Furthermore, as a result of this study, the observing doctors concluded, that of the  CVID patients exhibiting gastrointestinal symptoms, histological lesions were found in around 80% of the biopsies taken from the colon, stomach, or small bowel.

The enteropathy corresponding with CVID was found to have has many features that differentiate it from other etiopathological conditions including celiac disease. While replacement Ig therapy was demonstrated to be inadequate for improving gastrointestinal symptoms, steroids, specifically budesonide,were proven successful in reducing inflammation and restoring mucosal architecture.

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I was diagnosed in 2002 and I think I have had maybe 2 actual colds since then. I figured the same as you that with my immune system not having to try and 'save' me from gluten that it now is able to fight off the occasional virus. The only thing it hasn't been able to fight off is shingles. Thankfully those are clearing and I blame myself for that with lack of sleep and a very poor diet for a bit. Lesson learned, one does not live off crackers and cheese alone.

Welcome to the board. I agree with the previous posters that you are very likely looking at celiac. Please do keep her on gluten until all celiac related testing is finsihed. After that do give the diet a good strict try even if the biopsies are negative. Also keep in mind that celiac is genetic so it would be a good idea to screen others in the family even if they don't seem to have symptoms.

@jddh So...did the restricted diet you were going to implement work (FODMAP or Whole Foods)? I recall that you were mis-diagnosed at one point with refractory celiac disease, but it was later determined that you were getting trace amounts of gluten in your diet. If you are not catching colds, I assume that you have healed from the damages of celiac disease? I hope so!!! ?

Peter is correct. You do have a positive so that warrants further investigation. Here is a link supporting our comments: http://www.cureceliacdisease.org/faq/are-raised-dgp-igg-levels-an-early-sign-of-celiac-disease/ http://www.mayomedicallaboratories.com/it-mmfiles/Celiac_Disease_Diagnostic_Testing_Algorithm.pdf Does she have celiac disease? You will never know for sure without an endoscopy. Even then, there is a chance the biopsies are negative, but keep in mind that she might just be starting to develop celiac disease or that the damage was not captured (the small intestine is the size of a tennis court if spread out). Personally, I tested negative on all but the DGP IgA, yet I had moderate to severe intestinal damage. The celiac blood tests are good, but they do not catch all celiacs, some celiacs can even test negative to ALL the blood tests. Consider yourself fortunate that your doctor ordered several of the tests and not just the screening TTG IgA (very good, keeps cost down, but does not catch all). The DGP is the preferred test in small children. I do not know why it caught me because I am old, but it did! Confusing, isn't it? I wish there was an easier way to diagnose, but we have to work with what we have available to us.

Thank you for your reply, though it's not necessarily what I wanted to hear, it is what I was thinking.