[CIS PIDD] [cis-pidd] CVID and Shingles

[Seppänen Mikko]

Hi Michael,

I agree with all previous responses.

The easiest way for the clinician responsible for the patient to scan whether the patient has some CID instead of CVID might be to check
(this I have learned from prof Klaus Warnatz and Stephan Ehl/ Freiburg, yet again many thanks!)


1)      patient's naive CD3+CD4+ T cell counts (<200/ul?)/percentage of total (<10%)

2)      T gammadelta percentage (>11%).

Mitogen responses alone are not good, since they can be low/sluggish in CVIDs -
anyone else suggest a different, easier and cheaper approach?

Minor points:
As an ID specialist interested in herpesviruses, I can tell that it can just as well be HSV1/2 (even in CVIDs, CIDs). Especially if in lower back, buttocks, thighs, fingers or face.

Antigen/virus culture tests are best to take, since HSV is better suppressed with lower doses of vala, whereas usually VZV requires higher dose.
Since this will be long-term suppression, it is actually not a minor point for the patient nor fiscally.

HSV2 usually responds to Vala 1gx2 (even 500mgx2, at times), whereas VZV (and HSV1 if sunny) may need 1gx3. 500mgx1 almost never enough if PIDD (nor if very active disease in an immune competent, all? studies where 500mg dosing was established  excluded frequently active disease).
I often start with the highest dose and patient's can titrate to lowest effective and if needed increase the dose prophylactically when they know they are in risk (stress, stress released when holiday starts, sunshine, flus, etc,  highly individual). And breakthroughs are usually a sign of missed doses. If a drug resistant virus emerges, it usually never recurs (lesser fitness).

Vala is well tolerated long term even in these higher doses, but occasionally headache or nightmares (in less than 5%, in my experience), rarely stomach upset. And MCV, like in most antivirals, increases.

Mikko

dos Mikko Seppänen, LKT
Immuunipuutosv-o, HYKS

Mikko Seppänen, MD, PhD, Associate professor/Senior Lecturer
Specialist in Internal Medicine and Infectious Diseases
Senior Consultant, Physician in charge (PIDD)
Immunodeficiency Unit
Division of Infectious Diseases
Department of Medicine
Helsinki University Central Hospital
Hospital District of Helsinki and Uusimaa
Aurora Hospital, Ward 4-2 and Outpatient Clinic
P.O.Box 348
FI-00029 HUS, Helsinki
FINLAND
phone +358 9 47175923, fax +358 9 47175945






Lähettäjä: Prescott Atkinson, M.D. [mailto:PAtkinson at peds.uab.edu]
Lähetetty: 2. toukokuuta 2014 0:07
Vastaanottaja: CIS-PIDD
Aihe: RE: [cis-pidd] CVID and Shingles

Hi Mike:  I agree with other comments that this sounds like more than run-of-the-mill CVID and that prophylactic valtrex might be a better answer if recurrences have been frequent - has the virus been cultured from the lesions?  Recurrent HSV can look similar I think.  Are B cell and NK cell numbers normal?

If the pt is on IVIG, as far as I am aware, IVIG will neutralize/inactivate the vaccine (as with Varivax and MMR) for months out from the last dose - CDC recommends not giving for 8 mo .

Prescott


T. Prescott Atkinson, MD PhD, Professor and Director

Division of Pediatric Allergy, Asthma & Immunology

University of Alabama at Birmingham

Tel: 205-939-9072

Fax: 205-975-7080

________________________________
From: Riedl, Marc [mriedl at ucsd.edu]
Sent: Thursday, May 01, 2014 2:32 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] CVID and Shingles
Have seen recurrent documented shingles in a couple of CVID patients.  Restricted to dermatomes, but of considerable frequency, severity and duration.   I've advised against the shingles vaccine for my CVID patients and in these rare cases have opted for acyclovir or valacyclovir prophylaxis which has generally been effective.  ID colleagues have been agreeable to this approach.

Marc Riedl, MD, MS
Associate Professor of Medicine
Division of Rheumatology, Allergy & Immunology
University of California, San Diego
9500 Gilman Dr, Mail Code 0732
La Jolla, CA  92093
Tel 858.822.6766  Fax 858.642.3791

From: Klaus Warnatz <klaus.warnatz at uniklinik-freiburg.de<mailto:klaus.warnatz at uniklinik-freiburg.de>>
Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Date: Thursday, May 1, 2014 12:16 PM
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Subject: Re: [cis-pidd] CVID and Shingles

Dear Mike,

I don't have personal experience with shingles vaccination in CVID patients.
my thoughts on it would be, if the patient has recurrent shingles he might as well have some underlying cellular immunodeficiency.  I agree with Charlotte are these documented and what does recurrent mean? Are these outbreaks restricted to dermatomes at a time?
if not then it strongly suggests cellular immunodeficiency. I would be rather hesitant with vaccination and if documented and a substantial problem I would rather go for acyclovir prophylaxis.
Are there any other signs of cellular immunodeficiency? is she for any reason on immunosuppressive therapy? can one alter this?
what is her t cellular status?

These would be my thoughts

best regards

Klaus
Prof. Dr. med. Klaus Warnatz

UNIVERSITÄTSKLINIKUM FREIBURG
University Medical Center Freiburg
Center for Chronic Immunodeficiency
Division of Rheumatology and Clinical Immunology

Tel: +49-761-270-77640 / FAX -71000 / Pager: 12-7100

Breisacher Str. 117, 79106 Freiburg, Germany
klaus.warnatz at uniklinik-freiburg.de<mailto:klaus.warnatz at uniklinik-freiburg.de>
http://www.uniklinik-freiburg.de/cci

Am 01.05.2014 um 20:53 schrieb Cunningham-Rundles, Charlotte:


Is she on IVIG and is the shingles documented... ?


Charlotte Cunningham-Rundles, MD, PhD
Departments of Medicine and Pediatrics
The David S Gottesman Professor
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue
New York, NY 10029
Phone: 212 659 9268
Fax: 212 987 5593
Email: Charlotte.Cunningham-Rundles at mssm.edu<mailto:Charlotte.Cunningham-Rundles at mssm.edu>


From: Michael Blaese <mblaese at comcast.net<mailto:mblaese at comcast.net>>
Reply-To: "cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Date: Thursday, May 1, 2014 1:31 PM
To: "cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Subject: [cis-pidd] CVID and Shingles

IDF has received the following inquiry.
 "I have CVID and I get outbreaks of shingles. Has anyone had success with the shingles vaccination? Or is that too risky for someone with CVID?"

CDC still recommends against use of the vaccine in individuals with primary immunodeficiency.  Any thoughts or experience with this situation?

Mike

R. Michael Blaese, M.D.
Medical Director
Immune Deficiency Foundation


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