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

[Richard Wasserman]

I agree with Mary Ellen except that the choice between Asacol and Pentasa is
influenced by the location of maximum disease.
Richard Wasserman

On Fri, May 20, 2011 at 5:08 PM, Conley, Mary Ellen <
maryellen.conley at stjude.org> wrote:


>  I would guess that about 5% of patients with XLA have a GI disease that

> is like IBD.  Often the symptoms are preceded by an infection, but not

> always.  Like Richard Wasserman, we have found that steroids and 6MP or

> Immuran have been successful. Asacol is low risk and the GI doctors like it

> and so do I.  We have often added Flagyl, even when there is no documented

> infection.  It seems to help.  People are often reluctant to use adequate

> doses of steroids.  We go for 2 mg/kg prednisone and taper very slowly to an

> alternate day dose.  We don't try to taper them off for several years.  We

> have used parenteral nutrition in our sickest patient.  We have had relapses

> too.  Again, often preceded by an infection (giardia is the worst culprit!)

>

> We stay away from TNF inhibitors.  They seem to cause more problems.

>

> Mary Ellen

>

> Mary Ellen Conley, MD

> Department of Immunology/ Mail Stop 351

> St. Jude Children's Research Hospital

> 262 Danny Thomas Place

> Memphis, TN 38105-3678

> FAX  901-595-3977

> TEL  901-595-2576

>

>

>  ------------------------------

> *From:* pagid-bounces at list.clinimmsoc.org [mailto:

> pagid-bounces at list.clinimmsoc.org] *On Behalf Of *Richard Wasserman

> *Sent:* Friday, May 20, 2011 4:43 PM

>

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

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

>

>  I suspect every Pedi GI group has their preference but my patient was

> induced with prednisone and 6MP and has done very well. When 6MP was stopped

> several years ago he relapsed but was reinduced and has continued to do well

> on 6MP having weaned off steroids.

> Richard Wasserman

>

> On Fri, May 20, 2011 at 3:45 PM, Baxi, Sachin <

> Sachin.Baxi at childrens.harvard.edu> wrote:

>

>>  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

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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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>

>

>

> --

> 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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-- 
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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