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

[Jack Bleesing]

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 in this

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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

>>>> > Fax (972) 566-8837

>>>> > Cell (214) 697-7211

>>> 

>> 

> 

> 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

> 

> 

>