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

Thu Mar 30 01:40:05 EDT 2017

Thinking out loud:  Given his hypogammaglobulinemia, I would consider as etiologic agents a number of pathogens associated with arthritis: mycoplasma, parvovirus B19, EBV, rubella (less likely), and of course, think about a reactive arthritis following infection or colonization with Salmonella, Shigella, Camphylobacter or Yersinia species.  I would think mycoplasma or B19 are the most likely.  In adults, B19 can look like rheumatoid arthritis. Children tend to have the rash more prominently than joint involvement, but that is not always the case.  In adults, the synovium is histologically non-inflamed, but swelling and pain can still be present.  B19-induced tissue effects, including hepatitis, is mediated by transgene activation or apoptosis.  Consider checking serum for B19 DNA by PCR.  If he is B19 viremic, IVIG can be helpful in clearing the infection.  I would check for B19 and EBV DNA in serum before starting TNF therapy.  Does he have joint effusions that can be aspirated to look for inflammatory cells?

Stan

Stanley J. Naides, M.D., F.A.C.P., F.A.C.R.
Medical Director, Immunology R&D | Interim Scientific Director, Immunology R&D
Quest Diagnostics | Action from Insight | 33608 Ortega Highway| San Juan Capistrano, CA 92675| phone: 949-728-4578| fax: 949-728-7852
stanley.j.naides at QuestDiagnostics.com<mailto:stanley.j.naides at QuestDiagnostics.com>
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From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Wednesday, March 29, 2017 10:50 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] XLA with arthritis

Thank you Erwin!  I appreciate the wonderful comments.

Dave

________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Sent: Tuesday, March 28, 2017 2:58 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] XLA with arthritis

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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