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Peer-reviewedCC BYJournal of human immunity

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

  1. 1Division of Immunology and Allergy, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
  2. 2Division of Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
  3. 3Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Abstract

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.

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BibTeX
@article{ackerman2026nfkb1,
  title = {{NFKB1 truncation-associated recurrent, sterile inflammation effectively treated with IL-1β blockade.}},
  author = {Kevin S Ackerman and Isabel F Armendi and Jay J Mehta and Neil Romberg},
  journal = {Journal of human immunity},
  year = {2026},
  month = oct,
  doi = {10.70962/jhi.20260157},
  url = {https://europepmc.org/article/MED/42912666},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - NFKB1 truncation-associated recurrent, sterile inflammation effectively treated with IL-1β blockade.
AU  - Kevin S Ackerman
AU  - Isabel F Armendi
AU  - Jay J Mehta
AU  - Neil Romberg
PY  - 2026
DA  - 2026/10/23
JO  - Journal of human immunity
DO  - 10.70962/jhi.20260157
UR  - https://europepmc.org/article/MED/42912666
AB  - 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.
SN  - 3065-8993
ER  -