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Homozygous variant p. Arg90His in NCF1 is...
Journal article

Homozygous variant p. Arg90His in NCF1 is associated with early-onset Interferonopathy: a case report

Abstract

BackgroundBiallelic loss-of-function variants in NCF1 lead to reactive oxygen species deficiency and chronic granulomatous disease (CGD). Heterozygosity for the p.Arg90His variant in NCF1 has been associated with susceptibility to systemic lupus erythematosus, rheumatoid arthritis, and Sjögren’s syndrome in adult patients. This study demonstrates the association of the homozygous p.Arg90His variant with interferonopathy with features of …

Authors

Schnappauf O; Heale L; Dissanayake D; Tsai WL; Gadina M; Leto TL; Kastner DL; Malech HL; Kuhns DB; Aksentijevich I

Journal

Pediatric Rheumatology, Vol. 19, No. 1,

Publisher

Springer Nature

Publication Date

December 2021

DOI

10.1186/s12969-021-00536-y

ISSN

1546-0096