Immune disorders and crohns

[kimmidwife]

This is a really interesting article.
For our newer members, if your child has not had an immune work up it is important to ask your doctor to refer you to see an immunologist to rule out an immune disorder. There are a number of immune disorders where IBD is actually a secondary disorder and we have at least one member on the forum whose daughter was diagnosed with an immune disorder.
This article discusses how possibly all IBD is an immune disorder.

http://www.ncbi.nlm.nih.gov/pubmed/21997382

 

[Tesscorm]

Thanks.

Just curious, whether it is diagnosed as an immunodeficiency or impaired inflammatory response, would current treatment options change at all?

 

[kimmidwife]

Tess,
I think for some immune disorders they would add IVIG. Which is an IV infusion.

 

[CarolinAlaska]

Jae finally saw the immunologist on Thursday - a four hour visit! I'm sure it was her most comprehensive history ever taken. She has a bunch of labs to do this week.

 

[kiny]

Thanks.

Just curious, whether it is diagnosed as an immunodeficiency or impaired inflammatory response, would current treatment options change at all?

Not necessarily.

If you start thinking of crohn's disease as a primary immunodeficiency disease (which I believe it is), you're no longer talking about an autoimmune disease (which is a self-antigen response).

You're also no longer talking about IBD, because a term like IBD gives off the assumption that UC and Crohn's disease have some commonality. The article mentions IBD, but there is no convincing evidence that UC is an immunodeficiency disease, autophagy defects in ATG16L1, NOD2, and dysregulated cytokine and macrophage responses are only found in Crohn's disease.

It is very unlikely that UC is a primary immunodeficiency disease, it is very likely that crohn's is.


So, if crohn's disease is not like UC, or an autoimmune disease, what is it like? It would be similar to Chronic Granulomatous Disease (CGD). CGD is a primary immunodeficiency disorder, and the symptoms can be similar to crohn's disease. People with CGD develop CGD colitis.

People with CGD are treated with antibiotics, and sometimes anti-TNF.

 

[CrohnsDadNJ]

We just received the all clear from S' pediatric immunologist. It took a battery of tests as well as lots of questions about family history and child's history of infections. They look for a bunch of immune disorders but CGD in particular because it can masquerade/manifest as Crohn's, especially if biopsy has granulomas. The blood test to detect is tricky (requires a control sample from a non relative adult, sample cannot be refrigerated and needs to be air shipped to a specialized lab within 24 hours) and most regular lab technicians are unfamiliar with it. So we had to redo it a couple of times. CGD is treated with daily antibiotics, and long term outcomes are worse than Crohns.

 

[DanceMom]

Just wanted to add to this thread since A is diagnosed with an immune deficiency. She presents like classic Crohn's (chronic diarrhea, bloody stools, FTT, fatigue, erythema nodosum, etc.) so an immune deficiency wasn't even on the radar. She's never had pneumonia or strep throat which is typical of PID kids. Her IgG is low but IgA and IgM are normal. She builds an initial response to vaccines but titers fall after 4-6 months. She's not the norm, and I'm thankful our GI pushed for continued testing.

Be sure your immune workup included immunoglobulin levels and antibody titers. If titers were low and a vaccine challenge was needed be sure that levels are checked 6 months after vaccines were administered. Be sure that B cells and T cells were checked.

We don't know the exact PID that's causing A's Hypogammaglobulinemia, but IVIG has changed our world. For now we're just counting our blessings and enjoying our days.

 

[CrohnsDadNJ]

Hi DanceMom,

I looked at all the tests that the immunologist ran for S after I saw your message. Indeed, he got checked for immunoglobulin, B cells, T cells, DHR assay, the usual comprehensive metabolic panel as well as antibody titers for a whole host of vaccines/diseases. He was missing a few titers for pneumonia (all others vaccines were fine) so we are re-taking the pneumonia vaccine week. The immunologist also looked at the biopsies from the colonoscopy before giving the all clear.

The first result of DHR Assay were equivocal so we were afraid of CGD, but apparently it was caused by deterioration of sample in transit. Results were normal when we added a control sample in the second test and the doctor read the riot act to the lab on handling samples.

Hugs and blessings to you and your little one!

Just wanted to add to this thread since A is diagnosed with an immune deficiency. She presents like classic Crohn's (chronic diarrhea, bloody stools, FTT, fatigue, erythema nodosum, etc.) so an immune deficiency wasn't even on the radar. She's never had pneumonia or strep throat which is typical of PID kids. Her IgG is low but IgA and IgM are normal. She builds an initial response to vaccines but titers fall after 4-6 months. She's not the norm, and I'm thankful our GI pushed for continued testing.

Be sure your immune workup included immunoglobulin levels and antibody titers. If titers were low and a vaccine challenge was needed be sure that levels are checked 6 months after vaccines were administered. Be sure that B cells and T cells were checked.

We don't know the exact PID that's causing A's Hypogammaglobulinemia, but IVIG has changed our world. For now we're just counting our blessings and enjoying our days.

 

[my little penguin]

Glad you got the all clear in cgd
Ds was tested and it is very stressful to say the least

Did they send his blood out for the full genetic profile ?
I know cgd was the "easy test "
But the Gi wanted very specific genetic tests that mimic crohns and are specfic to early onset crohns
These take minimum of 3 months to process

 

[Malgrave]

COLORS in IBD study (COLitis of early Onset - Rare diseaseS withIN IBD)

http://www.ndm.ox.ac.uk/principal-investigators/researcher/holm-uhlig

 

[CrohnsDadNJ]

Glad you got the all clear in cgd
Ds was tested and it is very stressful to say the least

Did they send his blood out for the full genetic profile ?
I know cgd was the "easy test "
But the Gi wanted very specific genetic tests that mimic crohns and are specfic to early onset crohns
These take minimum of 3 months to process

No, we did not do any genetic tests. Our GIs (we have a primary and a secondary we use for second opinion) said that there are many genes implicated in Crohns and very few reliable genetic treatment approaches. They did recommend us participating in a few genetic research projects on VEO IBD at a couple of universities that we have contributed samples to. The immunologist was satisfied with the blood tests, the pathology results and the lack of history of infections; and said that we need not visit unless there is development of serious or recurrent infections.