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

[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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