[CIS-PAGID] (no subject)

[Kleiner, Gary]

I agree with Terri a similar patient responded to mmf after failing tnf and imuran by the gi service essentially all cells in gut were t cells 

Would be cautious about an allo stem cell. these patients often develop gvhd that is quite difficult to control 

Gary Kleiner MDPhD



On Feb 22, 2012, at 6:22 PM, "Terri Tarrant" <tarra002 at gmail.com> wrote:


> 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