Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition

Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition

Clinical application of several rapidly evolving technologies-next-generation DNA sequencing, biomarker discovery, and targeted cytokine blockade-has been particularly beneficial to understanding an expanding spectrum of genetically defined autoinflammatory diseases.1 Our understanding of the pathwa...

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Veröffentlicht in:Journal of allergy and clinical immunology 2017-05, Vol.139 (5), p.1698-1701
Hauptverfasser: Canna, Scott W., MD, Girard, Charlotte, MD, Malle, Louise, BS, de Jesus, Adriana, MD, PhD, Romberg, Neil, MD, Kelsen, Judith, MD, Surrey, Lea F., MD, Russo, Pierre, MD, Sleight, Andrew, PhD, Schiffrin, Eduardo, MD, Gabay, Cem, MD, Goldbach-Mansky, Raphaela, MD, MHS, Behrens, Edward M., MD
Format: Artikel
Sprache:eng
Schlagworte:
Allergy and Immunology
Bioindicators
Biological evolution
Cell activation
Cytotoxicity
DNA sequencing
Drug therapy
Hepatitis
Histiocytosis
Immunology
Inflammation
Inflammatory diseases
Inhibitor drugs
Interleukin 18
Lymphocytosis
Macrophages
Patients
Rheumatic diseases
Sepsis
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Zusammenfassung:Clinical application of several rapidly evolving technologies-next-generation DNA sequencing, biomarker discovery, and targeted cytokine blockade-has been particularly beneficial to understanding an expanding spectrum of genetically defined autoinflammatory diseases.1 Our understanding of the pathways that cause hemophagocytic disorders, such as macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH), is evolving similarly. MAS and HLH are life-threatening sepsis-like conditions notable for hyperferritinemia, acute cytopenias, and hepatitis. If not promptly recognized and treated, they can progress to consumptive coagulopathy, hemophagocytosis, multiorgan failure, and high mortality. HLH is classically associated with genetic defects in cytotoxicity, whereas MAS is observed as a complication of rheumatic diseases.1
ISSN:0091-6749
1097-6825
DOI:10.1016/j.jaci.2016.10.022