[CIS-PAGID] (no subject)

[Terri Tarrant]

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