Springer Seminars in Immunopathology

, Volume 27, Issue 4, pp 494–508

Hereditary auto-inflammatory disorders and biologics

  • Leigh D. Church
  • Sarah M. Churchman
  • Philip N. Hawkins
  • Michael F. McDermott
Original Article

DOI: 10.1007/s00281-006-0015-6

Cite this article as:
Church, L.D., Churchman, S.M., Hawkins, P.N. et al. Springer Semin Immun (2006) 27: 494. doi:10.1007/s00281-006-0015-6

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).

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Leigh D. Church
    • 1
  • Sarah M. Churchman
    • 1
  • Philip N. Hawkins
    • 2
  • Michael F. McDermott
    • 1
    • 3
  1. 1.Academic Unit of Musculoskeletal Disease, Leeds Institute of Molecular Medicine, Epidemiology and Cancer ResearchUniversity of LeedsLeedsUK
  2. 2.National Amyloidosis CentreRoyal Free & University College Medical SchoolLondonUK
  3. 3.Leeds Institute of Molecular Medicine, University of LeedsSt. James's University Teaching HospitalLeedsUK

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