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

Tue Mar 28 17:58:21 EDT 2017

As we reported, patients with XLA are very susceptible to mycoplasma, not the usual like M. pneumoniae but Urealyticum and others and may require intense antibiotics given susceptibility studies

Erwin W. Gelfand, M.D.
Department of Pediatrics
National Jewish Health
1400 Jackson Street
Denver, CO 80206
Ph: 303-398-1196
Fax: 303-270-2105
E-mail: gelfande at njhealth.org<mailto:gelfande at njhealth.org>

From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Tuesday, March 28, 2017 at 3:46 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto: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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