[CIS-PAGID] (no subject)

[Cunningham-Rundles, Charlotte]

Hi,

Just wondering if you tried Entocort, TID? Works in this much or most of the
time.  We also then switch to 6MP after a while, like in Crohns.

Charlotte

Charlotte Cunningham-Rundles, MD, PhD
Departments of Medicine and Pediatrics
The David S Gottesman Professor
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue
New York, NY 10029
Phone: 212 659 9268
Fax: 212 987 5593
Email: Charlotte.Cunningham-Rundles at mssm.edu





> From: <christian.wysocki at yale.edu>

> Reply-To: PAGID <pagid at list.clinimmsoc.org>

> Date: Thu, 23 Feb 2012 07:02:39 -0500

> To: PAGID <pagid at list.clinimmsoc.org>

> Subject: Re: [CIS-PAGID] (no subject)

> 

> Thanks again everybody.

> 

> The overarching themes seem to be

> 1.  Polytherapy, with T cell specific agents

> 2.  Further exploration of Treg deficiency (perhaps with FoxP3 staining), as

> Rapamycin may be worth adding to the arsenal if we find they are

> deficient, and

> it may suggest that she needs consideration of even more aggressive therapies

> such as transplant if these fail, although this would be high risk.

> 

> I will put these suggestions to the rest of the group.  Thanks very much,

> 

> Chris

> 

> 

> Quoting Terri Tarrant <tarra002 at gmail.com>:

> 

>> My most severe multiple-autoimmune CVID patient with villous atrophy

>> enteropathy who failed numerous immunosuppressive regimens has done

>> well on Cellcept 1000 mg PO bid. She had negative immunostaining for

>> CD19/CD20 in the gut despite peripheral B cells, and most of the

>> infiltrate was CD3+. I would caution using Rituximab if you only know

>> the flow results in the blood since they may be less representative

>> of IBD pathology, which in all types is more T cell driven. That

>> being said, I do have one patient with CVID/enteropathy/RA where it

>> worked in combination with methotrexate. :-)

>> 

>> Terri Tarrant

>> Rheum/AI UNC

>> 

>> Sent from my iPhone

>> 

>> 

>> On Feb 22, 2012, at 5:47 PM, 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

>> 

> 

>