Published in:
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. …