[CIS-PAGID] Duration of treatment for Crohn's in XLA

[Church, Joseph]

I agree with Dr. Blessing that the development of an auto immune-like
process should not be surprising in XLA.  Any compromise of the gut
mucosa barrier function may lead to pathologic inflammatory responses.
CGD is a good example.   The association of Crohns with XLA strongly
suggests that auto-antibody is not required for development of the
disease and that intervention for Crohns should be focused on innate
responses, T-cells or their cytokines.  

 

Joe Church

Children's Hospital Los Angele

________________________________

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Perez, Elena
Sent: Friday, April 08, 2011 9:34 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

 

In our patient path is also c/w Crohn, interestingly also presented
after a bout of fighting against colonization with C. jejuni. I am
willing to help get the data together and write up, but I don't know if
I have access to the registry...[my division is not yet "enrolled" in
the registry-but this could be the opportunity!?]

Elena

 

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Richard
Wasserman
Sent: Friday, April 08, 2011 12:29 PM
To: pagid at list.clinimmsoc.org
Cc: Jack Bleesing
Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

 

I my patient the pathology clearly looks like Crohn's. The onset was
more clearly post-infectious than is common for Crohn's I think. It may
be something else.
Richard

On Fri, Apr 8, 2011 at 11:14 AM, Jack Bleesing <Jack.Bleesing at cchmc.org>
wrote:

Question out of relative ignorance! Why the surprise? Is Crohn's purely
an autoimmune disease in the "classical" sense? And is it really Crohn's
disease in the classical sense?

J

-----Original Message-----
From: "Charlotte Cunningham-Rundles at mssm.edu"
<charlotte.cunningham-rundles at mssm.edu>
To: PAGID <pagid at list.clinimmsoc.org>
To: Kate <sullivak at mail.med.upenn.edu>

Sent: 4/8/2011 12:10:04 PM
Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

I was surprised too --- and have a good number of older ones!  It would
be a
neat question and could be answered!


On 4/8/11 12:03 PM, "Kate" <sullivak at mail.med.upenn.edu> wrote:


> I don't know if you are looking for research projects but you could

easily

> query the USIDNET registry and add all the patients that have been

discussed

> on PAGID.  Maybe I was the only one surprised but I suspect not.

>

> Kate

> On Apr 8, 2011, at 11:59 AM, Brian P Vickery wrote:

>

>> Kate, I was surprised to find autoimmunity in two of our XLA patients

at

>> Duke. I assumed the care of two young men, one in his early twenties,

and one

>> in his thirties, both of whom have XLA and ESRD due to an a

immune-mediated

>> kidney lesion like FSGS but with nonspecific pathology. The older one

also

>> had a very aggressive form of granulomatous Crohn's-like gut

inflammation

>> which had been treated with surgical resection in the past and more

recently

>> TNF inhibition.

>>

>> FWIW.

>> ___________________

>> Brian Vickery, MD

>> Division of Pediatric Allergy & Immunology

>> Duke University Medical Center

>> DUMC Box 2644, Durham NC 27710

>> 919.681.2949

>>

>> "Email should NOT be used for urgent medical issues. The information

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>>

>> -----pagid-bounces at list.clinimmsoc.org wrote: -----

>>

>>> To: pagid at list.clinimmsoc.org

>>> From: Kate Sullivan <sullivak at mail.med.upenn.edu>

>>> Sent by: pagid-bounces at list.clinimmsoc.org

>>> Date: 04/06/2011 05:54PM

>>> Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

>>>

>>> I don't know the answer to your question and was surprised by the

question.

>>> I think of XLA as being more free from autoimmune disease than other

immune

>>> deficiencies.  I wonder how common this is and wonder if this is

perhaps a

>>> topic for some research investigation.

>>>

>>> Kate

>>> Kate Sullivan, MD PhD

>>> Professor of Pediatrics

>>> ARC 1216 Immunology CHOP

>>> 3615 Civic Center Blvd.

>>> Philadelphia, PA 19104

>>> (p) 215-590-1697

>>> (f) 267-426-0363

>>>

>>>

>>> On Apr 6, 2011, at 5:27 PM, Richard Wasserman wrote:

>>>

>>>> > I have cared for this 23yo with XLA (BTK mutation confirmed) who

has a

>>>> ten year history of Crohn's. He has been maintained on azathioprine

and has

>>>> never been exposed to a biologic response modifier. When

azathioprine was

>>>> weaned about five years ago there was relapse. He has been doing

very well

>>>> since his remission was reinduced with prednisone. He now asks

about

>>>> stopping azathioprine. He has never had any other autoimmune

diathases. Is

>>>> there any experience with discontinuing treatment in XLA patients

with

>>>> Crohn's?

>>>> > Thanks,

>>>> > Richard Wasserman

>>>> > Dallas

>>>> >

>>>> > --

>>>> > Richard L. Wasserman, MD, PhD

>>>> > DallasAllergyImmunology

>>>> > 7777 Forest Lane, Suite B-332

>>>> > Dallas, Texas 75230

>>>> > Office (972) 566-7788 <tel:%28972%29%20566-7788> 

>>>> > Fax (972) 566-8837 <tel:%28972%29%20566-8837> 

>>>> > Cell (214) 697-7211 <tel:%28214%29%20697-7211> 

>>>

>>

>

> Kate Sullivan, MD PhD

> Professor of Pediatrics

> ARC 1216 Immunology CHOP

> 3615 Civic Center Blvd.

> Philadelphia, PA 19104

> (p) 215-590-1697

> (f) 267-426-0363

>

>

>





-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211



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