Anakinra for constrictive pericarditis associated with incessant or recurrent pericarditis

Objective: Frequent flares of pericardial inflammation in recurrent or incessant pericarditis with corticosteroid dependence and colchicine resistance may represent a risk factor for constrictive pericarditis (CP). This study was aimed at the identification of CP in these patients, evaluating the efficacy and safety of anakinra, a third-line treatment based on interleukin-1 inhibition, to treat CP and prevent the need for pericardiectomy. Methods: Consecutive patients with recurrent or incessant pericarditis with corticosteroid dependence and colchicine resistance were included in a prospective cohort study from 2015 to 2018. Enrolled patients received anakinra 100 mg once daily subcutaneously. The primary end point was the occurrence of CP. A clinical and echocardiographic follow-up was performed at 1, 3, 6 months and then every 6 months. Results: Thirty-nine patients (mean age 42 years, 67% females) were assessed, with a baseline recurrence rate of 2.76 flares/patient-year and a median disease duration of 12 months (IQR 9-20). During follow-up, CP was diagnosed in 8/39 (20%) patients. After anakinra dose of 100 mg/day, 5 patients (63%) had a complete resolution of pericardial constriction within a median of 1.2 months (IQR 1-4). In other three patients (37%), CP became chronic, requiring pericardiectomy within a median of 2.8 months (IQR 2-5). CP occurred in 11 patients (28%) with incessant course, which was associated with an increased risk of CP over time (HR for CP 30.6, 95% CI 3.69 to 253.09). Conclusions: In patients with recurrent or incessant pericarditis, anakinra may have a role in CP reversal. The risk of CP is associated with incessant rather than recurrent course.