[CIS PIDD] [cis-pidd] XLA with arthritis

[CIS-PIDD]

Others have talked about Flexispira and mycoplasma.  I would add enterovirus.  A small fraction of enterovirus in XLA patients presents as arthritis.
Finally, we and a few others have seen an LGL like process and that can have arthritis as a component.


Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363



On Mar 28, 2017, at 5:46 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Dear Colleagues,

I have a now 5 year old young man with XLA.  He originally had a history of recurrent OM and C.diff as well as occasional neutropenia.  He was referred for right knee swelling suggestive of JIA.   Based on his history additional studies documented data diagnostic of XLA.  No B cells.  Pan-hypogammaglobulinemia.  BTK expression studies and genetic analyses were done as well.  A hemizygous variant in BTK was documented.  I placed him on Hizentra and he has done wonderfully.  His arthritis also seemed to get better - he also had some corticosteroid injections.  More recently, he has developed a more significant flare of his JIA - more joints (wrists, knee, ankle, etc.).  We were going to use a anti-TNF agent (e.g. Humira or Enbrel).   I was wondering if anyone has any words of wisdom / caution in this setting given the risk of enterovirus issues, neutropenia....   any other suggested agents that would be excellent.

Thanks as always!

Dave

David Buchbinder, MD
CHOC Children's Hospital

---------------------------------------------------------------------
This email message and any files transmitted are sent with confidentiality in mind and contain privileged or copyright information. You must not present this message to another party without gaining permission from the sender. If you are not the intended recipient you must not copy, distribute or use this email or the information contained in it for any purpose other than to notify Children's Hospital of Orange County. Any views expressed in this message are those of the sender, except where the sender specifically states them to be the views of Children's Hospital of Orange County. If you have received this message in error, please notify the sender immediately, and delete this email from your system. We do not guarantee that this material is free from viruses or any other defects although due care has been taken to minimize the risk.
--------------------------------------------------------------------
---
You are currently subscribed to cis-pidd as: sullivank at email.chop.edu<mailto:sullivank at email.chop.edu>.
To unsubscribe click here: http://cts.dundee.net/u?id=99424140.0252c6305bcec2418e0f99fbf64e026c&n=T&l=cis-pidd&o=4291197
(It may be necessary to cut and paste the above URL if the line is broken)
or send a blank email to leave-4291197-99424140.0252c6305bcec2418e0f99fbf64e026c at lyris.dundee.net<mailto:leave-4291197-99424140.0252c6305bcec2418e0f99fbf64e026c at lyris.dundee.net>


---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=4291303
or send a blank email to leave-4291303-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20170328/690222b8/attachment-0001.html>