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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2013

Open Access 01-11-2013 | Meeting abstract

PW03-017 – Combination TNF and IL-1 blockade in PAPA syndrome

Authors: P Hoffmann, AK Ombrello, DL Stone, KS Barron, DL Kastner

Published in: Pediatric Rheumatology | Special Issue 1/2013

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Excerpt

The dominantly inherited PAPA syndrome is caused by mutations in PSTPIP1. It is one of the least understood monogenic autoinflammatory diseases, both from a pathogenic and treatment perspective. Patients often have persistent symptoms refractory to biologic therapies that necessitate prolonged exposure to high dose corticosteroids. Prior studies have reported increased production of IL-1b and TNF-α by peripheral blood leukocytes. There are Case reports documenting the efficacy of both IL-1 and TNF-α antagonists in treating PAPA but, to date, there are no reports of combination anti-TNF and anti-IL-1 use. …
Metadata
Title
PW03-017 – Combination TNF and IL-1 blockade in PAPA syndrome
Authors
P Hoffmann
AK Ombrello
DL Stone
KS Barron
DL Kastner
Publication date
01-11-2013
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue Special Issue 1/2013
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/1546-0096-11-S1-A243

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