Clinical Rheumatology

, Volume 33, Issue 9, pp 1197–1207 | Cite as

Key facts and hot spots on tumor necrosis factor receptor‐associated periodic syndrome

  • Donato Rigante
  • Giuseppe Lopalco
  • Antonio Vitale
  • Orso Maria Lucherini
  • Caterina De Clemente
  • Francesco Caso
  • Giacomo Emmi
  • Luisa Costa
  • Elena Silvestri
  • Laura Andreozzi
  • Florenzo Iannone
  • Mauro Galeazzi
  • Luca Cantarini
Review Article


Tumor necrosis factor receptor-associated periodic syndrome (TRAPS), formerly known as familial Hibernian fever, is the most common autosomal dominant autoinflammatory disease, resulting from mutations in the TNFRSF1A gene, encoding the 55-kD tumor necrosis factor receptor. The pathophysiologic mechanism of TRAPS remains ambiguous and only partially explained. The onset age of the syndrome is variable and the clinical scenery is characterized by recurrent episodes of high-grade fever that typically lasts 1–3 weeks, associated with migrating myalgia, pseudocellulitis, diffuse abdominal pain, appendicitis-like findings, ocular inflammatory signs, and risk of long-term amyloidosis. Fever episodes are responsive to high-dose corticosteroids, but different classes of drugs have been reported to be ineffective. The use of etanercept is unable to control systemic inflammation, while interleukin-1 blockade has been shown as effective in the control of disease activity in many patients reported so far.


Autoinflammatory disease Interleukin-1 inhibitors Tumor necrosis factor receptor‐associated periodic syndrome 


Conflict of interest



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Copyright information

© Clinical Rheumatology 2014

Authors and Affiliations

  • Donato Rigante
    • 1
  • Giuseppe Lopalco
    • 2
  • Antonio Vitale
    • 3
  • Orso Maria Lucherini
    • 3
  • Caterina De Clemente
    • 3
  • Francesco Caso
    • 3
    • 4
  • Giacomo Emmi
    • 5
  • Luisa Costa
    • 6
  • Elena Silvestri
    • 5
  • Laura Andreozzi
    • 1
  • Florenzo Iannone
    • 2
  • Mauro Galeazzi
    • 3
  • Luca Cantarini
    • 3
  1. 1.Institute of PediatricsUniversità Cattolica Sacro CuoreRomeItaly
  2. 2.Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic HospitalUniversity of BariBariItaly
  3. 3.Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le ScotteUniversity of SienaSienaItaly
  4. 4.Rheumatology Unit, Department of Medicine DIMEDUniversity of PaduaPaduaItaly
  5. 5.Department of Experimental and Clinical MedicineUniversity of FlorenceFlorenceItaly
  6. 6.Rheumatology Unit, Department of Clinical Medicine and SurgeryUniversity Federico IINaplesItaly

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