November 2025

RHEUMATOLOGY

CASE REPORT: IL-1 INHIBITORS FOR TRAPS-ASSOCIATED RECURRENT PERICARDITIS

A 26-year-old woman of Hispanic descent presented to Northwestern Medicine with persistent fever, rapid heart rate and pleuritic chest pain. She was diagnosed with recurrent pericarditis linked to TRAPS and achieved significant clinical improvement after transitioning from canakinumab to rilonacept.
Background
  • TRAPS: A hereditary autoinflammatory disorder caused by mutations in TNFRSF1A (TNF receptor 1 gene)
  • Clinical features: recurrent fever and systemic inflammation; pericarditis occurs in around~30% of cases

Case overview
  • Patient: 26-year-old Hispanic woman with recurrent fever, chest pain, dyspnea
  • Previous diagnosis: adult-onset Still’s disease (AOSD); multiple pericarditis episodes
  • Genetic testing: heterozygous TNFRSF1A variant (c.418C>A; p.Leu140Ile), likely linked to TRAPS

Management and outcome
  • Failed initial therapies included:
    • NSAIDs
    • colchicine
    • corticosteroids
    • canakinumab
  • Switched to rilonacept (IL-1α and IL-1β inhibitor):
    • Chest pain resolved
    • CRP normalized within 6 weeks
  • Plan: monitor markers, repeat cardiac MRI in 1 year; pericardiectomy only if biologic therapy fails

Key insights
  • RP often misclassified as idiopathic; consider autoinflammatory syndromes like TRAPS
  • IL-1 inhibitors (rilonacept > canakinumab) are effective for refractory RP
  • Genetic testing is valuable in persistent or atypical cases
  • Mutation inherited from mother; family history of autoimmune conditions

Conclusion
  • Autoinflammation is a major driver of RP.
  • IL-1 blockade offers targeted, effective therapy.
  • Genetic evaluation should be considered in refractory RP.
  • Radical pericardiectomy remains a last resort.
Arthur M. Mandelin, MD, PhD, Associate Professor of Rheumatology at Northwestern Medicine

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