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

[Baxi, Sachin]

Hello,
I see that there are a few cases of XLA and Crohn's disease.  I would appreciate any feedback on therapy regarding this severe case.

My patient is a 6 yo with XLA who had a severe drop in IgG about 2 years ago.  He had an ileitis which is this "Crohn's type" disease and, in spite of treatment, he progressed to stricturing of the small intestine and had two resections and an ileostomy this year.  Unfortunately, he had abrupt and prominent recurrence of disease in the small intestine a few weeks post operatively.

He is taking infliximab 10mg/kg q4weeks, prednisolone 1mg/kg/d, and will start IV methotrexate after an incomplete response to po methotrexate.  He has also taken antibiotics, liquid diet and a short course of thalidomide (which he did not tolerate).

The tissue samples are consistent with Crohn's disease.  There is no evidence of infection.  And tissue samples were negative for enterovirus (in regards to the NEJM article on regional enteritis assoc with enterovirus in a patient with XLA).

Since the resection, IgG troughs normalized to 900-1000 mg/dL on IVIG q 3 weeks.

Has anyone found a way to control the Crohn's disease?  This appears to be a very aggressive disease in XLA patients.

Thank you,
Sachin

Sachin Baxi, MD
Attending, Allergy and Immunology
Children's Hospital, Boston
Phone: 617-355-6117
Fax: 617-730-0310


________________________________
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Torgerson, Troy
Sent: Friday, April 08, 2011 3:12 PM
To: pagid at list.clinimmsoc.org; sullivak at mail.med.upenn.edu; Charlotte Cunningham-Rundles
Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

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

>>

>> "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 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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