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

[Perez, Elena]

I also have an AR agammaglobulinemic patient who recently was diagnosed with Crohn disease, initially responsive to steroids and pentasa, but now requiring remicaide. Patient also had occult bacteremia and stool cx positive with C. jejuni controlled with long term abx.  
Elena

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Howard Lederman
Sent: Wednesday, April 06, 2011 10:19 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

I have a patient with BLNK deficiency (I think the only BLNK deficient patient in the world)  that causes an XLA-like phenotype.  He has had a Crohn's like disease for 10 or 15 years.  (None of my XLA pts have Crohn's).



He has often been maintained only on Pentasa, but we have not been successful in weaning him off all meds.



Is he truly asymptomatic now?  Does he have a normal Hgb and MCV?  What is his CRP?  What is his stool alpha 1 anti-trypsin level?



Howard
Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics, Medicine and Pathology
Division of Pediatric Allergy and Immunology
Johns Hopkins Hospital - CMSC 1102
600 N. Wolfe Street
Baltimore, MD 21287-3923
Phone: 410-955-5883
Fax: 410-955-0229
Email: Hlederm1 at jhmi.edu<mailto:Hlederm1 at jhem.jhmi.edu>

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________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Richard Wasserman [drrichwasserman at gmail.com]
Sent: Wednesday, April 06, 2011 6:01 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

He was given a ferret by his home IV nurse and developed diarrhea the was positive for Giardia. he was treated, the Giardia eradicated and he never got better. On biopsy he had granulomata of the terminal ileum that was read out as Crohn's. He is my only XLA with chronic GI problems.
Richard

On Wed, Apr 6, 2011 at 4:54 PM, Kate Sullivan <sullivak at mail.med.upenn.edu<mailto:sullivak at mail.med.upenn.edu>> wrote:
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>





--
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