[CIS PIDD] [cis-pidd] Adult CVID, Recurrent Shingles

Wed Apr 26 13:24:20 EDT 2017

Joe -

I’ve seen a couple of CVID patients who suffer recurrent shingles.  They've shown some indication of cellular immunodeficiency (either lymphopenic or by functional studies).  I’ve not given VZV vaccine in these instances, but higher dose valacyclovir has been pretty effective.

Best,

Marc

Marc Riedl, MD, MS
Professor of Medicine
Adult Primary Immunodeficiency Program
Division of Rheumatology, Allergy & Immunology
University of California, San Diego
8899 University Center Lane, Suite 230
San Diego, CA  92122
Tel 858.657.5350  Fax 858.657.5375

From: <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>> on behalf of 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: Wednesday, April 26, 2017 at 7:03 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: RE: [cis-pidd] Adult CVID, Recurrent Shingles

Thank you, Charlotte.  I will have to get her in her the next time.  Joe Church

From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Wednesday, April 26, 2017 6:56 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] Adult CVID, Recurrent Shingles (EXTERNAL EMAIL)

Hi Joe,

Personally I think that H zoster is very (very)  rare in CVID pts on Ig therapy; my first impulse is to validate it with cultures.

Charlotte Cunningham-Rundles

On Apr 25, 2017, at 10:33 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Hi Joe,

I have not attempted, but most of these are in general HSV-1 recurrent whitlow, HSV-2 buttock/lower back lesions - or other similar site often in typical locations

Most of the breakthroughs are in HSV caused by
- either patient forgetting to take the medication regularly or
- by a temporal drug resistant mutant clone which however has a lesser fit for host and thus does not recur and does not establish dormancy in the ganglia.

So
- a single breakthrough: do nothing, emphasize regularity of medication
- recurrent breakthroughs: establish dg and if HSV, is the dose of valaciclovir large enough (up to 3g/daily)?
- if HSV, breakthroughs despite 3g/d OR if VZV
a) is CVID truly CVID? Highly frequent shingles makes me think of CID, a few occasional recurrences one does see in VZV and "CVID"
b) and in the case of HSV, is there even an impaired IFNg response, see Mihai Netea's group's article recently in JACI? However, patient would need to have to conditions, not exactly the Occam's razor

ATB,

Mikko Seppänen

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 25.4.2017 kello 22.03:
Colleagues:

I just started following an adult woman who was previously diagnosed with CVID.  She is doing very well with regards to respiratory and GI symptoms on weekly SCIg infusions.

However, she has experienced recurrent “shingles” and recently broke through valacyclovir prophylaxis.  The lesions have not been PCR’d for VZV.  She was told by another MD that a VZV vaccine may help.

I am a bit hesitant because she has a good VZV-specific lymphoproliferative response.

Assuming these are actually recurrent VZV outbreaks, has anyone noted an improvement in frequency or severity after administration of VZV vaccine?

Thank you for your time.

Joe Church
Children’s Hospital Los Angeles

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