Treatment of the Nephrotic Syndrome with Etanercept in Patients with the Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

Publisher Website
Google Scholar
Abstract
The tumor necrosis factor (TNF) receptor–associated periodic syndrome is a dominantly inherited, chronic inflammatory condition characterized by lifelong febrile attacks of abdominal and musculoskeletal pain associated with skin lesions. The attacks vary in severity and in frequency, usually last from 3 to 21 days, and respond to high-dose corticosteroids. Soluble TNF receptors can inhibit TNF activity by binding and preventing the interaction of TNF-α (TNFSF2) with its cell-surface receptor. Recombinant human TNF receptor (TNFRSF1B) (p75):Fc fusion protein (etanercept) inhibits the activity of TNF in vitro and reduces inflammation in animal models.1