Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes

Inessa Bachove,1 Christopher Chang2 1Cooper University Hospital, Children's Regional Hospital, Camden, NJ, USA; 2Division of Allergy and Immunology, Thomas Jefferson University, Wilmington, DE, USA Abstract: Anakinra is an interleukin (IL) receptor antagonist that works by blocking the bio...

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Autores principales:	Bachove I, Chang C
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2014
Materias:	Diseases of the musculoskeletal system RC925-935
Acceso en línea:	https://doaj.org/article/80ade94221284d4cbb9f27b629e96f10
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spelling	oai:doaj.org-article:80ade94221284d4cbb9f27b629e96f102021-12-02T05:34:12ZAnakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes1179-156Xhttps://doaj.org/article/80ade94221284d4cbb9f27b629e96f102014-03-01T00:00:00Zhttp://www.dovepress.com/anakinra-and-related-drugs-targeting-interleukin-1-in-the-treatment-of-a15989https://doaj.org/toc/1179-156X Inessa Bachove,1 Christopher Chang2 1Cooper University Hospital, Children's Regional Hospital, Camden, NJ, USA; 2Division of Allergy and Immunology, Thomas Jefferson University, Wilmington, DE, USA Abstract: Anakinra is an interleukin (IL) receptor antagonist that works by blocking the biological activity of IL-1 by competitively inhibiting binding of IL-1 to the type 1 interleukin receptor. IL-1 production is induced in response to inflammatory stimuli and mediates various physiological mechanisms, including inflammation and immunological reactions. Patients with neonatal onset multisystem inflammatory disease (NOMID) produce excess IL-1β, a major proinflammatory cytokine that regulates innate immune responses. Anakinra binds competitively and this results in a rapid reduction in disease severity. NOMID, also known as chronic infantile neurologic, cutaneous, articular syndrome, is the most severe clinical phenotype in the spectrum of cryopyrin-associated periodic syndromes. It is characterized by cutaneous symptoms, arthropathy, and central nervous system involvement. Extensive studies in patients with NOMID have led to advances in characterizing the extent of organ-specific involvement and damage that occurs with chronic overproduction of IL-1β. NOMID is caused predominantly by mutations in the NLRP3/CIAS1 gene that encodes for the protein cryopyrin, leading to activation of the “NLRP3 inflammasome complex”. This in turn regulates the maturation and secretion of the inflammatory cytokine, IL-1β. The clinical value of IL-1β has been demonstrated by the positive response of patients after treatment with anakinra, with rapid improvement in clinical symptoms, markers of inflammation, and a significant decrease in major organ manifestations. Keywords: neonatal onset multisystem inflammatory disease, chronic infantile neurologic, cutaneous, arthritis syndrome, NLRP3, CIAS1, interleukin-1βBachove IChang CDove Medical PressarticleDiseases of the musculoskeletal systemRC925-935ENOpen Access Rheumatology: Research and Reviews, Vol 2014, Iss default, Pp 15-25 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the musculoskeletal system RC925-935
spellingShingle	Diseases of the musculoskeletal system RC925-935 Bachove I Chang C Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes
description	Inessa Bachove,1 Christopher Chang2 1Cooper University Hospital, Children's Regional Hospital, Camden, NJ, USA; 2Division of Allergy and Immunology, Thomas Jefferson University, Wilmington, DE, USA Abstract: Anakinra is an interleukin (IL) receptor antagonist that works by blocking the biological activity of IL-1 by competitively inhibiting binding of IL-1 to the type 1 interleukin receptor. IL-1 production is induced in response to inflammatory stimuli and mediates various physiological mechanisms, including inflammation and immunological reactions. Patients with neonatal onset multisystem inflammatory disease (NOMID) produce excess IL-1β, a major proinflammatory cytokine that regulates innate immune responses. Anakinra binds competitively and this results in a rapid reduction in disease severity. NOMID, also known as chronic infantile neurologic, cutaneous, articular syndrome, is the most severe clinical phenotype in the spectrum of cryopyrin-associated periodic syndromes. It is characterized by cutaneous symptoms, arthropathy, and central nervous system involvement. Extensive studies in patients with NOMID have led to advances in characterizing the extent of organ-specific involvement and damage that occurs with chronic overproduction of IL-1β. NOMID is caused predominantly by mutations in the NLRP3/CIAS1 gene that encodes for the protein cryopyrin, leading to activation of the “NLRP3 inflammasome complex”. This in turn regulates the maturation and secretion of the inflammatory cytokine, IL-1β. The clinical value of IL-1β has been demonstrated by the positive response of patients after treatment with anakinra, with rapid improvement in clinical symptoms, markers of inflammation, and a significant decrease in major organ manifestations. Keywords: neonatal onset multisystem inflammatory disease, chronic infantile neurologic, cutaneous, arthritis syndrome, NLRP3, CIAS1, interleukin-1β
format	article
author	Bachove I Chang C
author_facet	Bachove I Chang C
author_sort	Bachove I
title	Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes
title_short	Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes
title_full	Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes
title_fullStr	Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes
title_full_unstemmed	Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes
title_sort	anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes
publisher	Dove Medical Press
publishDate	2014
url	https://doaj.org/article/80ade94221284d4cbb9f27b629e96f10
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Anakinra and related drugs targeting interleukin-1 in the treatment of cryopyrin- associated periodic syndromes

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