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Incidence of reactivated Herpes Zoster (shingles) in patients with Primary Immunodeficiency Diseases
Poster Title: Incidence of reactivated Herpes Zoster (shingles) in patients with Primary Immunodeficiency Diseases
Submitted on 09 Feb 2018
Author(s): David Lindsay MD, Charlotte Cunningham-Rundles MD, PhD, Ramsay L. Fuleihan MD, Mark Ballow, MD, Jennifer W. Leiding MD
Affiliations: Morsani College of Medicine University of South Florida, Icahn School of Medicine at Mount Sinai, Feinberg School of Medicine Northwestern University
This poster was presented at USF Research Day
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Poster Information
Abstract: Objective:
To determine the incidence and associated features of reactivated Herpes Zoster (shingles) in a cohort of patients with primary immune deficiency (PID).

Patients and Methods:
We submitted a query to the United States Immunodeficiency Network (USIDNET) to determine the incidence of shingles in their cohort. We received patient data from a 19 year period that spanned July 2, 1998 through January 18, 2017. The information included de-identified data including date of shingles infection, patient demographics, underlying diagnosis, and various other patient factors. We used this information to determine incidence and other factors associated with shingles infection.

From 1998 to 2017, a total of 183 patients in this cohort had an episode of shingles, with an overall incidence rate of 40 cases per 1,000 patients. Patient population was primarily Caucasian (91.8%) with median age of 39 years. 59% of patients were male and 41% were female. Patient with humoral primary immune deficiency were the highest proportion of the cohort. 48.6% of the patients who developed shingles had CVID and 8.2% had BTK deficiency. Interestingly, 15.8% of the patients had Wiskott-Aldrich syndrome.

Retrospective data from the USIDNET cohort suggest that there is an increased incidence of Varicella zoster in patients with PID. This incidence in this cohort is 10x higher than the average incidence rate in the US population of 4 cases per 1,000. It is also 4x higher than the incidence rate of 10 per 1,000 in patients aged 60 and older. A majority of patients had humoral immune deficiencies, however there was a broad spectrum of PIDs which had an episode of shingles. 76.5% of patients were on immunoglobulin supplementation, indicating that deficient cell-mediated immunity may also play an important role in the development of shingles in patients with PID.

Summary: Retrospective cohort study to determine incidence of Herpes Zoster in a large, multi-center cohort of patients with primary immunodeficiency.References: Ann M. Arvin; Humoral and Cellular Immunity to Varicella-Zoster Virus: An Overview, The Journal of Infectious Diseases, Volume 197, Issue Supplement_2, 1 March 2008, Pages S58–S60,
Christopher J.A. Duncan, Sophie Hambleton, Varicella zoster virus immunity: A primer, Journal of Infection, Volume 71, Supplement 1, 2015, Pages S47-S53,
Lesia K. Dropulic, Jeffrey I. Cohen; Severe Viral Infections and Primary Immunodeficiencies, Clinical Infectious Diseases, Volume 53, Issue 9, 1 November 2011, Pages 897–909,
Kawai K, Gebremeskel BG, Acosta CJ Systematic review of incidence and complications of herpes zoster: towards a global perspective BMJ Open 2014;4:e004833. doi: 10.1136/bmjopen-2014-004833
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