[CIS PIDD] [cis-pidd] infliximab for for CVID related enteropathy?

[Klaus Warnatz]

Dear Joseph, dear Elena, 

Did you exclude LRBA? XIAP? would the presentation be compatible with either one? other endocrinopathies? when was the onset of the enteropathy? hints for secondary lactose etc intolerance? signs of malabsorption?
Did your intensive work up of the enteropathy include Norovirus? we see chronic Norovirus infection in some of our patients, I assume the typical other culprits have been excluded? Do you have the HLA DQ2/8 typing? did you repeat gastroscopy recently? I would to confirm no change in the histopathology. 
we have used usually first budesonide when suspected AIenteropathy and need to apply it first with an capsule opened and taken together with mashed apple and then two capsules as they are later during the day. When this fails, systemic steroids and, if they fail and the histology shows clear T cell mediated disease, we usually use cyclosporine/tacrolimus or sirolimus. We have used anti TNF but only briefly without clear improvement. the London group has reported more success others have seen increased infections. Sufficient IgRT is important. 
Follow up: clinic, calprotectin in stool, gastroscopy 
I hope this helps. 

Klaus

Prof. Dr. med. Klaus Warnatz

UNIVERSITÄTSKLINIKUM FREIBURG
University Medical Center Freiburg
Center for Chronic Immunodeficiency
Division of Rheumatology and Clinical Immunology

Tel: +49-761-270-77640 / FAX -71000 / Pager: 12-7100

Breisacher Str. 117, 79106 Freiburg, Germany
klaus.warnatz at uniklinik-freiburg.de
http://www.uniklinik-freiburg.de/cci

Am 20.03.2014 um 05:34 schrieb Elena Hsieh:


> Hi there,

> 

> This e-mail is on behalf of my colleague Joseph Hernandez, he tried to e-mail the listserv but had trouble getting it through.  Thanks.

> Elena

> 

> Dear Colleagues,

> 

> We have a 21yo patient with CVID that we have been following for several years who has bad enteropathy characterized primarily by abdominal pain and profuse diarrhea. Biopsies that were done several years ago showed sprue like appearance lymphocyte infiltration, relatively few granulocytes and a virtual absence of plasma cells. Path did not show any evidence of CMV enteritis, and currently all infectious studies are negative. He has had partial improvement in his stools and abdominal pain in the past with steroids (methylpred, prednisone, or budesonide) but he is poorly compliant and has now had several relapses. We have not tried AZA or 6-MP. Our GI service is considering starting infliximab and methotrexate because he has not completely responded to steroids in the past and they feel it may be easier to monitor/ensure compliance. I am only able to find a few case reports of CVID enteropathy in the literature treated with anti-TNFs but I am sure that our community has more experience. Does anyone have much experience using anti-TNFs for enteropathy? Have you had many infectious complications?

> 

> A little more about the patient. He has very low IgGs (<200 on some occasions) and has had very low lymphocyte numbers (on multiple occasions CD4<200). He did have Tetanus specific proliferation and B cell phenotyping consistent with CVID. We have done an extensive workup for other causes of his immune deficiency and have not found any. He has had multiple sino/pulmonary infections in the past and has bronchiectasis as a result. He was diagnosed with hodgkins lymphoma a couple years ago, completed therapy and is now in remission. When he had a recurrence of pain and GI symptoms a couple months ago he underwent imaging, PET and Bone marrow bx that were negative. He also has type 1 DM that was diagnosed years before his CVID.

> 

> Thanks,

> 

> Joseph Hernandez

> Instructor, Immunology, Allergy & Rheumatology

> Stanford University

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