TNF Receptor Associated Periodic Syndrome (TRAPS) is a rare autoinflammatory disorder characterized by recurrent episodes of fever, abdominal pain, rash, and joint inflammation. It is caused by mutations in the TNFRSF1A gene, which encodes the tumor necrosis factor receptor 1 (TNFR1).
Due to its rarity, the prevalence of TRAPS is not well-established. However, it is estimated to affect approximately 1 in 1 million individuals worldwide. TRAPS can occur in both children and adults, with symptoms typically appearing in early childhood.
The condition is inherited in an autosomal dominant manner, meaning that a person with a mutation in one copy of the TNFRSF1A gene will develop TRAPS. However, not all individuals with the mutation will experience symptoms, leading to variability in disease expression.
Early diagnosis and appropriate management are crucial in TRAPS to prevent long-term complications and improve quality of life. Treatment options may include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and biologic agents targeting tumor necrosis factor (TNF) such as etanercept.
While TRAPS is a rare condition, it is important for healthcare professionals to be aware of its existence and consider it in the differential diagnosis of patients presenting with recurrent fever and other characteristic symptoms.