TRAPS: An Autosomal Dominant Autoinflammatory Syndrome

Abstract

Abstract

Hereditary periodic fever syndromes are genetic autoinflammatory disorders characterized by recurrent attacks of fever and inflammation. These diseases include familial Mediterranean fever (FMF), Tumor necrosis factor receptor-associated periodic syndrome (TRAPS), hyperimmunoglobulinemia-D with periodic fever syndrome (HIDS), neonatal onset multisystem inflammatory disorder/ chronic infantile neurological cutaneous and articular syndrome (NOMID/CINCA), familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS). TRAPS is an autosomal dominant autoinflammatory syndrome. The mutations in the gene encoding the Tumor Necrosis Factor Receptor Super Family 1A (TNFRSF1A) on chromosome 12p13 cause this disease. The clinical manifestations of TRAPS include recurrent fever, abdominal pain, localized myalgia, erythematous skin lesions, arthralgia, conjunctivitis, and periorbital edema. Glucocorticoids and etanercept may be effective in decreasing the severity, frequency and duration of symptoms in TRAPS patients.