TRAPS patients demonstrate exaggerated innate immune responsiveness and are characterized clinically by recurrent prolonged episodes of fever, migratory rash, peritonitis, myalgia and arthralgia, with systemic amyloidosis
- Also called familial Hibernian fever
- FHF resembles familial Mediterranean fever (FMF) in several clinical features, but the mode of inheritance of FHF is dominant whereas FMF is recessive.
- Mutations in the TNFRSF1A gene.
- Autosomal dominant pattern
- Codes for tumour necrosis factor receptor 1 (TNFR1).
- This binds to tumour necrosis factor (TNF).
- Binding causes inflammation or self-destruct and apoptosis.
- Recurrent episodes of fever last about 3 weeks
- Variable frequency weeks to years
- Triggers minor injury, infection, stress, exercise, or hormonal changes.
- Abdominal and muscle pain and a spreading skin rash
- Puffiness or swelling in the skin around the eyes (periorbital oedema)
- Joint pain.
- Inflammation of eyes, heart muscle, certain joints, throat, or mucous membranes such as the moist lining of the mouth and digestive tract.
- Secondary amyloidosis and renal failure.
- FBC, U&E : renal disease
- Elevated ESR
- Genetic testing
- Exclude FMF
- High serum IL-6 levels
- Familial Mediterranean fever
- Systemic vasculitis
- Consider nonsteroidal anti-inflammatory drugs or corticosteroids
- Antibodies against
Antibody therapy against
- TNF using etanercept. It is effective in some patients but this may become less effective with time
- Interleukin-1 using anakinra and canakinumab (Ilaris). These medications are usually helpful even in those patients who do not respond well to etanercept.
- Interleukin-6 using tocilizumab have also been helpful