title: Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition.
authors.affiliation: Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Md. Electronic address: scott.canna@chp.edu.
authors.forename: Scott W
authors.initials: SW
authors.lastname: Canna
authors.affiliation: Division of Rheumatology, Department of Internal Medicine Specialties, University Hospitals of Geneva, Geneva, Switzerland.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Girard
authors.affiliation: Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Md.
authors.forename: Louise
authors.initials: L
authors.lastname: Malle
authors.affiliation: Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Md.
authors.forename: Adriana
authors.initials: A
authors.lastname: de Jesus
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, Pa.
authors.forename: Neil
authors.initials: N
authors.lastname: Romberg
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, Pa.
authors.forename: Judith
authors.initials: J
authors.lastname: Kelsen
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, Pa.
authors.forename: Lea F
authors.initials: LF
authors.lastname: Surrey
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, Pa.
authors.forename: Pierre
authors.initials: P
authors.lastname: Russo
authors.affiliation: AB2Bio, Ltd, Lausanne, Switzerland.
authors.forename: Andrew
authors.initials: A
authors.lastname: Sleight
authors.affiliation: AB2Bio, Ltd, Lausanne, Switzerland.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Schiffrin
authors.affiliation: Division of Rheumatology, Department of Internal Medicine Specialties, University Hospitals of Geneva, Geneva, Switzerland.
authors.forename: Cem
authors.initials: C
authors.lastname: Gabay
authors.affiliation: Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Md.
authors.forename: Raphaela
authors.initials: R
authors.lastname: Goldbach-Mansky
authors.affiliation: Children's Hospital of Philadelphia, Philadelphia, Pa.
authors.forename: Edward M
authors.initials: EM
authors.lastname: Behrens
mesh_terms: D015551:Autoimmunity / Q000235:genetics; D053468:CARD Signaling Adaptor Proteins / Q000235:genetics*; D002135:Calcium-Binding Proteins / Q000235:genetics*; D004760:Enterocolitis / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D005260:Female; D020869:Gene Expression Profiling; D006801:Humans; D007223:Infant; D058847:Inflammasomes / Q000235:genetics; D007249:Inflammation / Q000235:genetics; D036341:Intercellular Signaling Peptides and Proteins / Q000494:pharmacology / Q000627:therapeutic use*; D020382:Interleukin-18 / Q000037:antagonists & inhibitors; D055501:Macrophage Activation Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D009154:Mutation / Q000235:genetics*; D010252:Paramyxoviridae / Q000276:immunology*; D018184:Paramyxoviridae Infections / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D011110:Polymorphism, Genetic; D016896:Treatment Outcome; D000073359:Exome Sequencing
chemical_list: D053468:CARD Signaling Adaptor Proteins; D002135:Calcium-Binding Proteins; D058847:Inflammasomes; D036341:Intercellular Signaling Peptides and Proteins; D020382:Interleukin-18; C435692:NLRC4 protein, human; C118370:interleukin-18 binding protein