Tumor necrosis factor receptor 1 associated periodic syndrome

Tumor necrosis factor receptor 1 associated periodic syndrome, most commonly known by its abbreviation TRAPS, is a rare inherited condition that causes repeated episodes of fever and inflammation throughout the body. It used to be called 'familial Hibernian fever' because it was first described in families of Irish and Scottish descent, though we now know it affects people of all backgrounds. TRAPS is caused by changes (mutations) in a gene called TNFRSF1A, which controls a protein that helps regulate the body's inflammatory response. When this gene does not work properly, the immune system becomes overactive and triggers episodes of fever and inflammation that can last days to weeks at a time. During a flare, people with TRAPS may experience high fevers, severe abdominal pain, chest pain, muscle aches, skin rashes, and swelling around the eyes. These episodes can be very disabling and unpredictable. Between attacks, many people feel relatively well, though some have ongoing low-level inflammation. Treatment has improved significantly in recent years. Anti-inflammatory medications, including corticosteroids, have long been used to shorten attacks. Biologic therapies that target specific parts of the immune system — especially a drug called canakinumab (Ilaris), which is FDA-approved for TRAPS — have been a major advance. Another biologic, etanercept, is sometimes used but with variable results. The main long-term concern is a complication called amyloidosis, where inflammatory proteins build up in organs, particularly the kidneys. With proper monitoring and treatment, many people with TRAPS can manage their symptoms and reduce this risk.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.Which specific mutation do I have in the TNFRSF1A gene, and what does that mean for my risk of complications like amyloidosis?,Should my family members be tested for the same mutation?,Is canakinumab (Ilaris) the right treatment for me, and how will we know if it is working?,How often should I have blood and urine tests to check for kidney damage or amyloidosis?,What should I do at home when a flare starts — is there a rescue treatment plan?,Are there any clinical trials I should know about for TRAPS?,What are the long-term risks if my inflammation is not fully controlled?

Common questions about Tumor necrosis factor receptor 1 associated periodic syndrome