familial hibernian fever (FHF); tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS)

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Epidemiology

  • age at onset:
    • 50% < 10 years of age
    • up to age 50 years
  • no ethnic predilection

Genetics

Clinical manifestations

Laboratory

Differential diagnosis

Management

More general terms

References

  1. UniProt http://www.uniprot.org/uniprot/P19438.html
  2. McDermott MF et al Germline mutations in the extracellular domains of the 55 kDa TNF receptor, TNFR1, define a family of dominantly inherited autoinflammatory syndromes. Cell 97:133-44, 1999 PMID: https://www.ncbi.nlm.nih.gov/pubmed/10199409
  3. Trost S & Rose CD Myocarditis and sacroilitis: 2 previously unrecognized manifestations of tumor necrosis factor receptor-associated periodic syndrome. J Rheumatol 2005; 32:175 PMID: https://www.ncbi.nlm.nih.gov/pubmed/15630744
  4. 4.0 4.1 4.2 4.3 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18. American College of Physicians, Philadelphia 2012, 2015, 2018.
  5. 5.0 5.1 De Benedetti F, Gattorno M, Anton J et al Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes. N Engl J Med 2018; 378:1908-1919. May 17, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29768139 https://www.nejm.org/doi/full/10.1056/NEJMoa1706314
  6. NEJM Knowledge+ Complex Medical Care

Database