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

[Notarangelo, Luigi]

We have also recently seen very severe Crohn's disease in a patient with molecularly proven XLA. Kate suggested to run a study and possible report these cases, and I think it would be a good idea!

Gigi Notarangelo
Children's Hospital Boston

Sent from my Verizon Wireless BlackBerry

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From: Brian P Vickery <brian.vickery at duke.edu>
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Date: Fri, 8 Apr 2011 11:59:24 -0400
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Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

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
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To: pagid at list.clinimmsoc.org
From: Kate Sullivan <sullivak at mail.med.upenn.edu>
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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

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