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

[Notarangelo, Luigi]

Yes, and there are data that suggest that TLR-signaling is abnormal in non-B cells from XLA (Sediva et al in Blood)

Gigi

Sent from my Verizon Wireless BlackBerry

________________________________
From: "Church, Joseph" <JChurch at chla.usc.edu>
Sender: "pagid-bounces at list.clinimmsoc.org" <pagid-bounces at list.clinimmsoc.org>
Date: Fri, 8 Apr 2011 13:10:32 -0400
To: pagid at list.clinimmsoc.org<pagid at list.clinimmsoc.org>
ReplyTo: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

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<mailto: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<mailto:Cunningham-Rundles at mssm.edu>" <charlotte.cunningham-rundles at mssm.edu<mailto:charlotte.cunningham-rundles at mssm.edu>>
To: PAGID <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>
To: Kate <sullivak at mail.med.upenn.edu<mailto: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<mailto: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<tel:919.681.2949>

>>

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

>> electronic mail is sensitive, protected information intended only for the

>> addressee(s). Any other person, including anyone who believes he/she might

>> have received it due to an addressing error, is requested to notify the

>> sender immediately by return electronic mail, and to delete it without

>> further reading or retention. The information is not to be forwarded to or

>> shared unless in compliance with Duke Medicine policies on confidentiality

>> and/or with the approval of the sender."

>>

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

>>

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

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

>>> Sent by: pagid-bounces at list.clinimmsoc.org<mailto: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<tel:215-590-1697>

>>> (f) 267-426-0363<tel: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<tel:215-590-1697>

> (f) 267-426-0363<tel: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

---------------------------------------------------------------------
CONFIDENTIALITY NOTICE: This e-mail message, including any attachments,
is for the sole use of the intended recipient(s) and may contain confidential
or legally privileged information. Any unauthorized review, use, disclosure
or distribution is prohibited. If you are not the intended recipient, please
contact the sender by reply e-mail and destroy all copies of this original message.
---------------------------------------------------------------------