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Seminars in Immunopathology
Published in: Seminars in Immunopathology 4/2006

01-06-2006 | Original Article

Hereditary auto-inflammatory disorders and biologics

Authors: Leigh D. Church, Sarah M. Churchman, Philip N. Hawkins, Michael F. McDermott

Published in: Seminars in Immunopathology | Issue 4/2006

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Abstract

The term auto-inflammatory disorders has been coined to describe a group of conditions characterized by spontaneously relapsing and remitting bouts of systemic inflammation without apparent involvement of antigen-specific T cells or significant production of auto-antibodies. The hereditary periodic fever syndromes are considered as the prototypic auto-inflammatory diseases, and genetic studies have yielded important new insights into innate immunity. DNA analysis has greatly enhanced the clinical characterization of these conditions, and elucidation of their molecular aetiopathogenesis has suggested that therapies may be aimed at specific targets within the immune cascade. The availability of biologic response modifiers such as inhibitors of tumour necrosis factor (TNF) and interleukin-1β has greatly improved the outlook for some of these disorders, although effective therapies remain elusive in patients with certain conditions, including hyperimmunoglobulinaemia-D with periodic fever syndrome (HIDS) and a proportion of those with TNF-receptor associated periodic syndrome (TRAPS). Indeed, outstanding challenges and the unique potential to further elucidate molecular mechanisms in innate immunity are illustrated by the dashed early hope that TNF blockade would be a panacea for TRAPS: not only is etanercept (Enbrel) ineffective in some cases, but there are anecdotal reports of this condition being greatly exacerbated by infliximab (Remicade).
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Metadata
Title
Hereditary auto-inflammatory disorders and biologics
Authors
Leigh D. Church
Sarah M. Churchman
Philip N. Hawkins
Michael F. McDermott
Publication date
01-06-2006
Publisher
Springer-Verlag
Published in
Seminars in Immunopathology / Issue 4/2006
Print ISSN: 1863-2297
Electronic ISSN: 1863-2300
DOI
https://doi.org/10.1007/s00281-006-0015-6

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