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

[CIS-PIDD]

Several years ago Joe Church reported a case of HLA B27(-) enthesitis in a
young man with XLA which responded beautifully to chronic NSAID therapy.  I
encountered a very similar case recently: recurrent hospital admissions for
suspected septic arthritis involving the knees and ankles, cultures and
Ureaplasma PCR uniformly negative.  No recurrences since starting daily
naproxen 6 months ago.

Hope that helps.

Robert

Robert W. Sugerman, M.D.


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On Tue, Mar 28, 2017 at 4:46 PM, CIS-PIDD <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
>
>
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