Clinical Rheumatology

, Volume 25, Issue 6, pp 773–777

TNF-receptor-associated periodic syndrome (TRAPS): an autosomal dominant multisystem disorder

Authors

    • Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
    • Division of Genomic MedicineThe University of Sheffield
    • Immunology, Asthma and Allergy Research InstituteChildren Medical Center
Review

DOI: 10.1007/s10067-005-0198-6

Cite this article as:
Rezaei, N. Clin Rheumatol (2006) 25: 773. doi:10.1007/s10067-005-0198-6

Abstract

The TNF-receptor-associated periodic sydrome (TRAPS) is an autosomal dominant auto-inflammatory disorder, characterized by recurrent febrile attacks and localized inflammation. TRAPS is caused by mutations in the gene encoding the TNF Receptor Super Family 1A (TNFRSF1A) on chromosome 12p13. However, the incomplete penetrance and genetic heterogeneity have been reported in this syndrome. Although the ethnic diversity and clinical heterogeneity may propose the role of other genes in the pathogenesis of TRAPS, some low-penetrance TNFRSF1A variants contribute to atypical inflammatory responses in other autoimmune diseases. Furthermore, molecular studies on TRAPS and other auto-inflammatory disorders could be suggested to identify additional genes coding the molecules in the TNF signalling process.

Keywords

Hereditary periodic fever syndromesMutationPenetranceTNFRSF1A (Tumor necrosis factor receptor superfamily, member 1A)

Copyright information

© Clinical Rheumatology 2006