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

Epidemiology: - age at onset: - 50% < 10 years of age - up to age 50 years - no ethnic predilection Genetics: - associated with defects in TNFRSF1A - autosomal dominant disease Clinical manifestations: - recurrent fever - abdominal pain, serositis - localized tender skin lesions - migratory rash [4] - myalgia - pleurisy - myocarditis - sacroiliitis - arthritis of large joints - conjunctivitis - periorbital edema - attacks last days to weeks [4] generally 7-21 days [] Laboratory: - complete blood count (CBC): leukocytosis - high ESR Differential diagnosis: - familial Mediterranean fever - episodes of fever & serositis generally last 1-3 days - asymmetric arthritis: knees, ankles & wrists - no conjunctivitis or periorbital edema Management: - glucocorticoid - TNF-alpha inhibitor [4] - canakinumab (Ilaris) may be helpful in controlling flares [5]

General

hereditary periodic fever syndrome; periodic fevers; recurrent fever syndrome; familial autoinflammatory disease

Database Correlations

OMIM 142680

References

  1. UniProt :accession P19438
  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: 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: 15630744
  4. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18. American College of Physicians, Philadelphia 2012, 2015, 2018.
  5. 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: 29768139 https://www.nejm.org/doi/full/10.1056/NEJMoa1706314
  6. NEJM Knowledge+ Complex Medical Care