PW02-013 - The role of IL6 and LPS in pathogenesis of TRAPS

S Savic,Mf Mcdermott,Ch Wong,W Miriam,L Ouboussad,R Doble,Lj Dickie

doi:10.1186/1546-0096-11-s1-a153

Abstract

Tumour necrosis factor (TNF) receptor– associated periodic syndrome (TRAPS) is a hereditary autoinflammatory condition resulting from a range of mutations in the TNFRSF1A gene. It is characterised in part by recurrent episodes of inflammation, affecting connective tissues, and manifesting as migratory erysipelas, myalgia and serositis. A number of aberrant inflammatory responses have been described in this condition, including hyperresponsiveness to lipopolysaccharide (LPS). The microRNA mir155 has been implicated in mediating downstream pro-inflammatory response to LPS, and it has has also been implicated in the pathogenesis of rheumatoid arthritis (RA). Tocilizumab (anti-IL6 receptor antibody) has been reported to be an effective treatment in at least one published case of TRAPS.

Highlights

Tumour necrosis factor (TNF) receptor– associated periodic syndrome (TRAPS) is a hereditary autoinflammatory condition resulting from a range of mutations in the TNFRSF1A gene
The microRNA mir155 has been implicated in mediating downstream pro-inflammatory response to LPS, and it has has been implicated in the pathogenesis of rheumatoid arthritis (RA)
The patient’s fibroblasts showed greater production of IL-6 in response to LPS and IL-1 but not TNF when compared to healthy control (HC)

Summary

Introduction

Tumour necrosis factor (TNF) receptor– associated periodic syndrome (TRAPS) is a hereditary autoinflammatory condition resulting from a range of mutations in the TNFRSF1A gene. It is characterised in part by recurrent episodes of inflammation, affecting connective tissues, and manifesting as migratory erysipelas, myalgia and serositis. A number of aberrant inflammatory responses have been described in this condition, including hyperresponsiveness to lipopolysaccharide (LPS). The microRNA mir155 has been implicated in mediating downstream pro-inflammatory response to LPS, and it has has been implicated in the pathogenesis of rheumatoid arthritis (RA). Tocilizumab (anti-IL6 receptor antibody) has been reported to be an effective treatment in at least one published case of TRAPS

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Results