NFKB1 truncation–associated recurrent, sterile inflammation effectively treated with IL-1β blockade

Recurrent, sterile inflammatory responses to surgery and trauma in a patient with a heterozygous, truncating NFKB1 variant are described. This case illustrates the autoinflammatory phenotype of truncating NFKB1 variants and highlights the prospect of a targeted, personalized immunomodulatory therapy.