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

[Prof. Dr. Antonio Condino Neto]

A current hypothesis tells that cytokines are destroyed in macrophages  
in CD, and these cells are unable to control microrganisms in GI tract.

Perhaps BTK or a BTK-dependent molecule may have a role on cytokine  
expression in macrophages.
-- 
Antonio Condino-Neto
Professor of Experimental Medicine
Institute of Biomedical Sciences, University of São Paulo
1730 Lineu Prestes Avenue, São Paulo - SP. ZIP 05508-000. Brazil
Tel (55) (11) 3091-7387 / Fax (55) (11) 3091-7224



Citando "Torgerson, Troy" <troy.torgerson at seattlechildrens.org>:


> We have/had 3 patients with XLA and significant IBD that has been  

> labeled as "Crohn's" disease - Jack's question of whether it is  

> really Crohn's disease is a good one.  In general, the bowel disease  

> does not improve with IVIG, even combining IVIG with oral IgG (yes,  

> we tried it).  It does seem to improve with other immune suppression  

> that is often used to treat IBD such as azathioprine but we have  

> been very worried about suppressing T cell immunity after one of our  

> patients developed degenerative CNS disease and was subsequently  

> found to have astrovirus in the brain tissue.  Once the bowel  

> disease starts, our experience has been that it is very difficult to  

> get patients off their immune suppression because the bowel disease  

> revs up again as meds are tapered.

>

> Hans has told me a number of times that before the etiology of XLA  

> was known, he used to do rectal suction biopsies on all of the XLA  

> kids and virtually all of them had crypt abscesses even in the  

> absence of overt gut disease - they often used this as one of the  

> diagnostic clues.  It is intriguing to think about what might be  

> different in the pathophysiology and histopathology of patients with  

> bowel disease caused by lack of Tregs (IPEX) vs. that caused by lack  

> of B cells (XLA) vs. that caused by lack of functional neutrophils  

> (CGD).

>

> TT

>

> Troy R. Torgerson, MD PhD

>

> Assistant Professor, Pediatric Immunology/Rheumatology

> Co-Director Immunology Diagnostic Laboratory (IDL)

> University of Washington & Seattle Children's Hospital

> Center for Immunity and Immunotherapies

> Seattle Children's Research Institute

> 1900 9th Ave., C9S-7

> Seattle, WA  98101-1304

>

> Tel:  (206) 987-7450

> Fax:  (206) 987-7310

>

>

>

>

>

> On 4/8/11 9:14 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

>>>

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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

>>

>>

>>

>

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