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Familial Mediterranean fever (FMF) and beyond: a new horizon. Fourth International Congress on the Systemic Autoinflammatory Diseases held in Bethesda, USA, 6–10 November 2005
  1. S Ozen1,
  2. H M Hoffman2,
  3. J Frenkel3,
  4. D Kastner4
  1. 1Department of Paediatrics, Hacettepe University, Ankara, Turkey
  2. 2Department of Paediatrics, University of California at San Diego, La Jolla CA, USA
  3. 3Department of General Paediatrics, University Medical Centre Utrecht, The Netherlands
  4. 4National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda MD, USA
  1. Correspondence to:
    Professor S Ozen
    Department of Paediatrics, Hacettepe University, Ankara 06100 Turkey; sezaozen{at}


Autoinflammatory diseases are characterised by seemingly unprovoked inflammation. They can be categorised as: hereditary (monogenic) autoinflammatory diseases, complex (polygenic/multifactorial) autoinflammatory diseases, and diseases where the course is affected by mutations in the defined autoinflammatory disease genes. Identification of the inflammatory pathways involved has opened up new areas of research which have implications for the treatment of these disorders and the pathogenesis of common inflammatory diseases.

  • ASC, apoptosis associated speck-like protein with a caspase-recruitment domain
  • CAPS, cryopyrin associated periodic syndromes
  • CINCA, chronic infantile neurological cutaneous articular syndrome
  • FCAS, familial cold autoinflammatory syndrome
  • FMF, familial Mediterranean fever
  • HIDS, hyper immunoglobulin D syndrome
  • IL, interleukin
  • MEFV, gene for Mediterranean fever
  • MWS, Muckle-Wells syndrome
  • NOMID, neonatal onset multisystem inflammatory disease
  • PAMPs, pathogen associated molecular patterns
  • PAPA, pyogenic arthritis with pyoderma gangrenosum and acne
  • PFAPA, periodic fever adenopathy pharyngitis and aphthous stomatitis
  • PSTPIP1, proline serine threonine phosphatase-interacting protein 1
  • SoJIA, systemic onset juvenile idiopathic arthritis
  • TNF, tumour necrosis factor
  • TRAPS, tumour necrosis factor associated periodic syndrome
  • familial Mediterranean fever
  • periodic fever syndromes

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