[CIS PIDD] [cis-pidd] XLA+UC

[Church, Joseph]

Thank you all for your very helpful comments and suggestions.  JC

From: Conley, Mary Ellen [mailto:mconley at uthsc.edu]
Sent: Friday, February 01, 2013 7:51 AM
To: CIS-PIDD
Subject: RE:[cis-pidd] XLA+UC

I think I have commented on inflammatory bowel disease in XLA before in this forum.  It is not all that uncommon.  I would guess that it occurs in about 5-10% of patients with XLA and maybe a little higher percentage of patients with autosomal recessive agamma.  It often starts after an infection, giardia is the most common but I have seen it after campylobacter, enterovirus and cryptosporidium.  These infections sometimes go unnoticed because they don't cause the typical symptoms.  The clues are lower serum IgG than expected on therapy, poor growth and some malaise.  I would recommend an aggressive search for an infection and addition of Flagyl even if you don't find giardia.  You need to treat with steroid at the same doses you would use in IBD and give it for a long time.   We have often added azathioprine as steroid sparing.  I would avoid TNF inhibitors as they tend to make things worse (maybe because of a background of infection?).  Animal studies suggest that normal B cells in the gut produce IL-10 and this decreases the inflammatory T cell response after an infection.  This may explain what is going on in XLA.
Mary Ellen

Mary Ellen Conley, MD
West Research Tower
LeBonheur Children's Hospital
50 N. Dunlap St.
Memphis TN  38103-2800
Tel     901-287-4657
FAX  901-287-4551
mconley at uthsc.edu<mailto:mconley at uthsc.edu<mailto:mconley at uthsc.edu%3cmailto:mconley at uthsc.edu>>

From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Thursday, January 31, 2013 9:32 PM
To: CIS-PIDD
Subject: [cis-pidd] XLA+UC

Colleagues:

An 8yo boy with XLA was just diagnosed as having "ulcerative colitis."

I could find no reference to this specific association.

Has anyone seen this combination?

Should I recommend a second Pathology opinion?

Joe Church
Children's Hospital Los Angeles

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