Abstract 18975: First Reported Case of SLE Associated Recurrent Pericarditis Treated With Rilonacept

Abstract

Description of case: A 42 year old female presented with a 1 month history of exertional dyspnea and intermittent pleuritic chest pain. She had a 12-year history of systemic lupus erythematosus (SLE) and non-specific interstitial pneumonitis being managed with mycophenolate mofetil. Labs showed an ESR of 76 mm/hr and CRP of 5.5 mg/dL. CT chest ruled out pulmonary embolism but showed a moderate pericardial effusion, also seen on an echocardiogram. A cardiac MRI (cMRI) showed moderate diffuse circumferential pericardial enhancement. She was diagnosed with lupus pericarditis and initially treated with ibuprofen, colchicine, prednisone, and hydroxychloroquine; however, continued to have persistent symptoms over the next few months. Repeat cMRI showed a transition to effusive constrictive physiology. She was offered pericardiectomy but following shared decision making and multi-disciplinary discussion, she was started on Rilonacept. She experienced significant and sustained relief in symptoms, improvement in inflammatory markers and reduction of pericardial enhancement on cMRI. Discussion To the best of our knowledge, this is the first case of Rilonacept used to successfully treat recurrent pericarditis from SLE. Recurrence in chronic idiopathic pericarditis is thought to be driven by the innate immune system and Interleukin-1 (IL-1), while in autoimmune diseases, it is mediated by the adaptive immune system and Type 1 Interferon, which is associated with a poorer prognosis. The RHAPSODY and AIRTRIP trials demonstrating efficacy of IL-1 blockers in recurrent pericarditis excluded patients with autoimmune diseases. Use of IL-1 blockers to treat Lupus pericarditis has only been reported in 2 cases using Anakinra. Our patient’s positive response to Rilonacept suggests the presence of additional target pathways in autoimmune diseases that may mediate pericarditis recurrence. Future prospective studies are needed to elucidate this further.