[CIS PIDD] [cis-pidd] Rubella in granulomas

[CIS-PIDD]

Tony,
We are following a 5 year old with AT and(virtually) absent IgG and IgA with elevated IgM, progressive cutaneous granulomatous skin disease, positive for rubella.Her T cell function was reasonably good including DTH responses. She also carries a homozygous TAP mutation but HLA expression was normal.
I think given the number of AT patients and other PID, that these patients with granulomas may have a second hit, which is what we are working on.
We tried a variety of topical therapies and systemic steroids and are now beginning Humira. The rubella is puzzling in terms of the link to the skin lesions and why AT (although other PID have it as well).
Kate Sullivan has some ideas related to CD8 epitopes but the failure to isolate live virus poses some issues as to infection potential.
If you succeed with any therapy let us know.




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: Thursday, January 12, 2017 at 1:16 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: [cis-pidd] Rubella in granulomas

Greetings,

I am caring for an 18 y/o male pt. with ataxia-telangiectasia who has exuberant cutaneous and hepatic granulomas which (both) stain positive for rubella capsid. I am looking for possible therapies to consider on the assumption that the presence of the virus has some role to play in pathogenesis. I am not aware that this has been “proven” in any case, which is why I call it an assumption. Things to consider:


1.       High dose IgG. While probably not effective by itself and there is some evidence to suggest lack of efficacy for established infection, it could be an adjunct to something else.

2.       Something else:

a.       Interferon – type 1, I have heard second-hand anecdotal report regarding a lack of efficacy but would be interested in hearing from someone who has tried it.

b.      Interferon gamma, not sure if anyone has tried it

c.       Ribavirin, I have heard anecdotally that it is being tried but I don’t know about results

d.      Nitizoxanide, same

I would appreciate everyone’s thoughts, thank you.

Tony B.

Francisco A. Bonilla, M.D., Ph.D.
Director, Clinical Immunology Program, Boston Children’s Hospital
Associate Professor of Pediatrics, Harvard Medical School


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