Treatment of tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS)

IL: interleukin; NSAID: nonsteroidal antiinflammatory drug; TNF: tumor necrosis factor; TNFRSF1A: TNF receptor superfamily member 1A.

* Features of a typical attack include one or more of the following: fever lasting ≥7 days, myalgia, migratory rash, periorbital edema, and abdominal pain.

¶ Typical length of treatment with an antiinflammatory dose of an NSAID such as naproxen or ibuprofen is 3 to 5 days.

Δ Some patients may need higher doses of prednisone or prednisolone (eg, 1 mg/kg twice daily) or may respond well to a lower initial dose (0.5 mg/kg/day).

◊ Frequent attacks are arbitrarily defined as more than 3 to 4 attacks per year.

§ Risk factors for amyloidosis include evidence of ongoing inflammation (frequent attacks, poor response of attacks to oral glucocorticoids, persistent elevation of inflammatory markers between attacks) and TNFRSF1A mutations classified as pathogenic or likely pathogenic.

¥ Starting dose of canakinumab is 2 mg/kg/dose (maximum dose 150 mg) subcutaneously once every 4 weeks. If the initial response is inadequate, the dose may be repeated after 7 days and subsequent maintenance doses may be increased to 4 mg/kg/dose (maximum dose 300 mg) every 4 weeks.

‡ Treatment failure is defined as inadequate symptom control (frequency and/or severity of attacks), persistent elevation of inflammatory markers, or evidence of amyloidosis.