To determine the incidence and associated features of reactivated Herpes Zoster (shingles) in a cohort of patients with primary immune deficiency diseases (PIDD).
A query was submitted to the United States Immunodeficiency Network (USIDNET) to determine the incidence of shingles in their cohort. Data from a 19 year period (1998-2017) was analyzed for history of shingles infection, PIDD diagnosis, demographics, and characteristics of immune phenotype.
A total of 183 patients had an episode of shingles, with an overall incidence of 40 cases per 1,000 patients. Patient population was primarily Caucasian (91.8%) with median age of 39 years and male predominance (59% male, 41% female). Patients with humoral primary immune deficiency were 61.2% of the cohort. Forty eight percent of patients who developed shingles had common variable immune deficiency, which represents 5.6% of all patients with CVID in the USIDNET registry. Wiskott-Aldrich syndrome was the most common diagnosis in patients without humoral immune deficiency, comprising 15.9% of the patients in this query.
Retrospective data from the USIDNET cohort suggest that there is an increased incidence of Varicella zoster in patients with PIDD. The incidence in this cohort is 10x higher than the average incidence rate in the US population of 4 cases per 1,000 and 4x higher than the incidence rate of 10 per 1,000 in adults aged 60 and older. A majority of patients in this cohort had humoral immune deficiencies, indicating that these patients may be a particularly high risk for development of shingles.