[CIS-PAGID] (no subject)

[christian.wysocki at yale.edu]

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

>