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

[CIS-PIDD]

Thanks Prescott!  We just did some basic cultures.  We will obtain additional synovial fluid and look for Mycoplasma species by PCR....  do you know who will do 16S rRNA sequencing to look for Mycoplasma?


Dave


________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Tuesday, March 28, 2017 3:35 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] XLA with arthritis


There are many reports of arthritis in antibody deficient patients caused by mycoplasmas.  We are following a young woman who had untreated CVID who developed a destructive arthritis in her left foot, knee and right wrist that proved to be due to Mycoplasma salivarium by culture and 16S/NGS sequencing.



Prescott





From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, March 28, 2017 4:46 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: Re:[cis-pidd] XLA with arthritis



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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