PW03-017 – Combination TNF and IL-1 blockade in PAPA syndrome
نویسندگان
چکیده
Introduction 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-a by peripheral blood leukocytes. There are Case reports documenting the efficacy of both IL-1 and TNF-a antagonists in treating PAPA but, to date, there are no reports of combination anti-TNF and anti-IL-1 use.
منابع مشابه
Use of TNF inhibitors in the treatment of PAPA syndrome
Introduction PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum and acne syndrome) is a rare autoinflammatory disease caused by mutations in the PSTPIP1 gene. This disease is difficult to treat, but the combination of prednisone, an IL-1 inhibitor and a TNF-inhibitor has, in our experience, helped even the most severely affected patients. Treatment with anakinra appears to prevent most of ...
متن کاملTrials Organisation (PRINTO) and the Eurofever/Eurotraps Projects". Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review. ANNALS OF THE RHEUMATIC DISEASES
Objective To evaluate the response to treatment of autoinflammatory diseases from an international registry and an up‐to‐date literature review. Methods The response to treatment was studied in a web‐based registry in which clinical information on anonymised patients with autoinflammatory diseases was collected retrospectively as part of the Eurofever initiative. Participating hos...
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Introduction Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an autoinflammatory disorder of childhood and little is known about the underlying etiology. While mutations involving the IL-1 pathway have been identified in other recurrent fever disorders, including TNF-receptor associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)...
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