A Toolkit for Health Care Professionals Recurrent Pericarditis corticosteroids within six to 10 weeks following IL-1 receptor blocker commencement. Colchicine should be maintained and withdrawn only as the last drug after achieving stable remission. It has the safest cardiovascular profile and is now proposed to help prevent major adverse cardiac events (MACE) in patients with atherosclerotic cardiovascular disease. In patients who achieve stable remission with a chronic low dose of corticosteroids (e.g., prednisone ≤5 mg or equivalent) plus colchicine, the decision to switch to IL-1 blocker therapy should be individualized, taking into account factors such as tolerability, age, sex-related considerations and patient preference. Assess patients for persistence of disease by suspending treatment. It should be noted that IL-1 receptor blocker therapy discontinuation, while the underlying disease is still present, is associated with a high risk of pericarditis recurrence. In the International Registry of Anakinra for Pericarditis (IRAP), anakinra discontinuation was associated with a 57% risk of recurrence at 36-month follow-up.79 Table 6: Dosing of IL-1 blockers in recurrent pericarditis. Rilonacept14 FDA-approved* Adults Loading dose: 320 mg (divided into two 160 mg doses administered subcutaneously at different injection sites). Maintenance dose: 160 mg subcutaneously once weekly, starting one week after initial dose. Pediatric Patients (12 to 17 years old) Loading dose: 4.4 mg/kg, up to a maximum of 320 mg, delivered as one or two injections (not to exceed 2 mL/injection). Maintenance dose: 2.2 mg/kg subcutaneously, up to a maximum of 160 mg, once weekly. Anakinra13 Dose: 1–2 mg/kg, up to a maximum of 100 mg, subcutaneously every day. Consider dosing every other day in patients with creatinine clearance less than 30 mL/min. Azathioprine and intravenous immunoglobulin may be considered after the failure of IL-1 inhibitors and steroids, however prospective clinical trial evidence is limited. *Rilonacept is the only FDA-approved therapy for the treatment of recurrent pericarditis and reduction in risk of recurrence in adults and pediatric patients 12 years and older. Time to pericarditis recurrence (approximately two months) after rilonacept suspension without taper is predicted by the gradual washout pharmacokinetics of once-weekly rilonacept. Rilonacept suspension during the long-term extension phase of the RHAPSODY study was associated with a recurrence rate of 75% with a median time to recurrence of 11.8 weeks.80 In a retrospective review of 17 Italian patients after RHAPSODY completion, approximately 80% experienced a pericarditis recurrence after rilonacept suspension, with median time to recurrence of eight weeks. Potential strategies to reduce the risk of pericarditis recurrence following IL-1 receptor blocker discontinuation include longer duration of IL-1 receptor blocker therapy and medication tapering. Due to a longer elimination half-life (about seven days), RHAPSODY used a simpler paradigm of treatment cessation without tapering, enabled by the gradual washout of rilonacept over approximately eight weeks. In a sub-analysis of RHAPSODY assessing patient-reported outcomes in patients with recurrence, the return of recurrent pericarditis symptoms was gradual, with pain scores increasing in the one- to two-week period prior to a reported event. Reinitiation of rilonacept resulted in the resolution of the acute recurrence in patients where the underlying disease was still present. The effectiveness of changing the FDA-approved interval of rilonacept administration on subsequent pericarditis recurrence risk is unknown. Potential tapering strategies for anakinra81 • Reduce by one administration day per week. • Reduce to every other day dosing for three months, followed by half dose every other day for one to three months. 23 Potential strategies to reduce the risk of pericarditis recurrence following IL-1 receptor blocker discontinuation include longer duration of IL-1 receptor blocker therapy and medication tapering.
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