Inherited IFNAR1 deficiency in otherwise healthy patients with adverse reaction to measles and yellow fever live vaccines

We report two otherwise healthy patients, a 9-yr-old boy in Iran with severe measles vaccine disease at 1 yr and a 14-yr-old girl in Brazil with viscerotropic disease caused by the YF vaccine at 12 yr. The Iranian patient is homozygous and the Brazilian patient compound heterozygous for loss-of-function IFNAR1 variations. Patient-derived fibroblasts are susceptible to viruses, including the YF and measles virus vaccine strains, in the absence or presence of exogenous type I IFN. The patients’ fibroblast phenotypes are rescued with WT IFNAR1. Autosomal recessive, complete IFNAR1 deficiency can result in life-threatening complications of vaccination with live attenuated measles and YF viruses in previously healthy individuals.

Source: The Journal of Experimental Medicine - September 1, 2019 Category: Internal Medicine Authors: Hernandez, N., Bucciol, G., Moens, L., Le Pen, J., Shahrooei, M., Goudouris, E., Shirkani, A., Changi-Ashtiani, M., Rokni-Zadeh, H., Sayar, E. H., Reisli, I., Lefevre-Utile, A., Zijlmans, D., Jurado, A., Pholien, R., Drutman, S., Belkaya, S., Cobat, A., B Tags: Immunodeficiency, Innate Immunity and Inflammation, Infectious Disease and Host Defense Articles Source Type: research