Methods: A retrospective chart review was conducted on 3 patients, aged 32-42 years, with confirmed RRP lasting 2-12 years.
Results: Case 1 is a 32 year old female with familial Mediterranean fever and a 12-year history of RRP unresponsive to steroids and colchicine. Serum IL1-β levels were elevated (69.6 pg/mL, normal <3.9), and treatment with IVIG and abortive Anakinra resulted in a clinical improvement. Case 2 is a 42 year-old female with hypogammaglobulinemia, lupus, Sjogren syndrome, and a three-year history of RRP despite treatment with glucocorticoids, colchicine, and toradol. IL1-β was markedly elevated (137.5 pg/mL). Case 3 is a 38 year-old male with chronic EBV infection and a three-year history of RRP unresponsive to colchicine and steroids. IL1-β was elevated (5 pg/mL).
Conclusions: This case series describes three patients with recurrent refractory pericarditis who were found to have elevated IL1-β, one of whom responded to Anakinra, an IL-1-receptor antagonist. These cases indicate that IL1-β may be implicated in the pathogenesis of recurrent refractory pericarditis and suggest a potential role for Anakinra in the management of this disease.