REFRACTORY PERICARDITIS TREATED WITH CONCURRENT SUBCUTANEOUS IMMUNOGLOBULIN AND INTERLEUKIN 1 INHIBITOR THERAPY

Introduction Patients with refractory pericarditis have been treated with intravenous immunoglobulin or interleukin 1 (IL1) receptor antagonist (Anakinra) with limited success. Separate or combined therapy with subcutaneous immunoglobulin (HyQvia) and IL1 inhibitor (Rilonacept) for refractory pericarditis has not been previously described. Case Description The patient is an Italian male who was previously well until 16 years of age when he presented with pericarditis and elevated coxsackie B titers. He had 6 episodes of pericarditis over 6 years, despite maintenance oral corticosteroids and azathioprine. Immunologic work-up was notable for elevated IL1β (39.8 pg/mL, range + B cells (29 cells/μL, range 180-492 cells/μL). The patient had no evidence of underlying autoimmunity or periodic fever syndrome. He was started on HyQvia and then Anakinra was added. The patient was transitioned from Anakinra to Rilonacept for ease of administration. Oral corticosteroids and then azathioprine were slowly weaned. 2 years after starting HyQvia and Rilonacept, the patient is asymptomatic. Discussion This patient had evidence of immunodeficiency and autoinflammatory disease contributing to his pericarditis. Recurrent pericarditis has multifactorial etiology causing immune dysregulation. Treatment with HyQvia and Rilonacept was highly effective in this patient with documented elevation of serum IL1β, and superior to corticosteroid and azathioprine therapy.