Tumor Necrosis Factor Receptor–Associated Periodic Syndrome
Open Access
- 1 December 2000
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of Dermatology
- Vol. 136 (12) , 1487-1494
- https://doi.org/10.1001/archderm.136.12.1487
Abstract
TUMOR NECROSIS factor receptor–associated periodic syndrome (TRAPS) is a disorder characterized by prolonged episodes of fever and local inflammation caused by dominantly inherited mutations in TNFRSF1A, the gene encoding the 55-kDa tumor necrosis factor (TNF) receptor.1 This nomenclature subsumes several older diagnoses, including familial Hibernian fever (FHF),2,3 benign autosomal dominant familial periodic fever,4 and autosomal dominant periodic fever with amyloidosis,5 all of which share a common molecular basis. In addition to TRAPS, there are 2 other well-characterized inherited periodic fever syndromes: familial Mediterranean fever (FMF)6,7 and the hyperimmunoglobulinemia D syndrome (HIDS) with periodic fever.8,9 Although these syndromes have recurring episodes of fever accompanied by cutaneous and other inflammatory manifestations, they are caused by mutations in different genes.Keywords
This publication has 1 reference indexed in Scilit:
- Pyrin/marenostrin mutations in familial Mediterranean feverQJM: An International Journal of Medicine, 1998