[CIS-PAGID] (no subject)

[Christine M. Seroogy, MD]

Dear Christian,

Have you looked at Treg cells in your patient?

See the following report, your patient has some similar features:

http://www.sciencedirect.com/science/article/pii/S1081120610602517

Chris

Christine M. Seroogy MD, FAAAAI
Associate Professor
University of Wisconsin
Department of Pediatrics
Division of Allergy, Immunology & Rheumatology
1111 Highland Avenue
4139 WIMR
Madison, WI  53705-2275
phone: 608-263-2652
fax: 608-265-9721

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On 2/22/12 4:47 PM, "christian.wysocki at yale.edu"
<christian.wysocki at yale.edu> wrote:


>We have a really sick young lady, 24 years old with CVID, enteropathy and

>other

>autoimmune sequelae.  Her enteropathy has been severe for many years and

>refractory to numerous immunomodulatory therapies as detailed below.  She

>has

>required TPN since september 2011.  We are hoping to get opinions as to

>any

>therapies for severe, refractory CVID associated enteropathy, that people

>have

>had success with...we are particularly interested in Rituximab, and

>wondered

>what peoples experience with it were, but are open to any and all

>suggestions. 

>Please see a more detailed clinical description below.

>

>Severe chronic diarrhea since age 3.  Biopsies in the past showed a

>celiac-like

>process with villous blunting in the small bowel and lymphocytic

>infiltrates

>(primarily CD3+....don't know CD4 or 8) in small and large bowel.  She

>failed a

>gluten-free diet, and was somewhat improved on prednisone and Imuran.

>

>In adolescence, she developed a bad pneumonia, and recurrent sinusitis,

>and was

>referred to us when total IgG, A and M were found to be undetectable

>(total IgG

>had been 1500, IgA <7 when tested at age 3).  In the meantime, she had

>developed type 1 diabetes and JRA.  She has been on Ig replacement

>since...made

>more difficult by GI protein loss causing difficulty maintaining troughs.

> She

>is now on Hizentra and doing better from that perspective. We have done

>numerous flow analyses over the years....initially B total B cells were

>quite

>low (27/ul), but those have come up to normal more recently.  We did a

>detailed

>analysis 2 years ago, which showed almost no class switched memory B

>cells,

>normal CD21.

>

>The GI process still rages out of control, such that she has been on TPN

>since

>September.  The GI docs have tried numerous immunomodulatory agents over

>the

>years which seem to provide only transient, if any, benefit, including

>oral

>steroids (which she is now on chronically), Imuran, cyclosporin, Remicade,

>Humira and most recently, Cimzia.  A 6 month trial of Cimzia has failed.

>

>Thanks for any help or advice you can provide,

>- Chris Wysocki, Yale Allergy and Immunology fellow