Successful Treatment with Infliximab of a Patient with Tumor Necrosis Factor-associated Periodic Syndrome (TRAPS) Who Failed to Respond to Etanercept

To the Editor: Tumor necrosis factor (TNF)-associated periodic syndrome (TRAPS) is an autosomal dominant autoinflammatory disorder, previously known as Hibernian fever1, renamed TRAPS following the elucidation of TNFRSF1A as the susceptibility gene2,3, with mutations occurring generally on chromosome 12p13. The condition is autoinflammatory as there is no involvement of antigen-specific T cells, pathogens, or antibody production. TRAPS is characterized by recurrent febrile attacks of > 1 week duration, severe abdominal pain, myalgia, rash, conjunctivitis, headache, periorbital edema, and pleuritis4. Treatment options for TRAPS have met with limited success. Nonsteroidal antiinflammatory drugs are beneficial in controlling fever, but not abdominal and musculoskeletal symptoms. Glucocorticoids, although effective for inflammatory symptoms, do not prevent future attacks and the use of escalating doses is limited by toxicity5. Anti-TNF agents have demonstrated variable efficacy5–14. We describe the case of a 48-year-old woman of Italian Catalonian descent with TRAPS who was treated successfully with infliximab. At disease onset in 1994, our patient presented with fevers lasting 7–14 days with monthly periodicity, increasing to twice monthly 3 years later. With the attacks, she experienced recurrent migratory rashes that were tender, red, raised, papular, and nodular, and serpinginous and annular plaques over her body including face and trunk (Figures 1A and 1B). She had recurrent oral ulcers, sore throat, arthralgias, arthritis with knee effusions, myalgias, pleuritic-like chest pain, abdominal pain, eye redness and swelling, and debilitating fatigue. In 1998, attacks became almost continuous, with only 7 days of symptom-free relief per month. She could no longer function at her occupation as a photography laboratory receptionist. Figure 1. TRAPS-associated cutaneous lesions prior to treatment with infliximab (A, lateral view; B, posterior view). Acute-phase reactants were only modestly elevated during attacks (Figure 2). Antinuclear antibodies, rheumatoid … Address correspondence to Dr. Chaiton; E-mail: achaiton{at}aol.com

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.