Info
TNF receptor-associated periodic syndrome
- TNF receptor mutations → febrile episodes q 5-6 wks each lasting >7 d w/ myalgia, abd pain/nausea, migratory rash, periorbital edema; risk of amyloidosis (AA)
- Epidemiology: autosomal dom. w/ variable penetr.; prev 1/million; onset usually as child
- Rx: steroid taper; NSAID alone if mild flare. If freq or severe flares, anti-IL1 (canakinumab) induction and maintenance (NEJM 2018;378:1908) appears more effective than anti-TNF