IL1-Beta Levels in Patients with Refractory Recurrent Pericarditis
Sunday, March 6, 2016
South Exhibit Hall H (Convention Center)
Rushita Mehta, MD, Arye Rubinstein, MD
Rationale: Recurrent pericarditis is a debilitating condition thought to have an underlying immunologically-mediated mechanism. This condition is often resistant to conventional therapeutic options including non-steroidal anti-inflammatory drugs, colchicine, and glucocorticoids. We report three cases of refractory recurrent pericarditis (RRP) who were found to have elevated serum IL1-β levels, one of whom responded to Anakinra, an IL1-receptor antagonist.  These findings implicate IL1-β in RRP pathogenesis and suggest a therapeutic role for IL1-β blockage.

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.