Abstract 16644: Unusual Manifestation of Acute Pericarditis in the Setting of Primary Biliary Cirrhosis

Introduction: Primary biliary cirrhosis (PBC) is an immune-mediated liver disease leading to destruction of intrahepatic bile ducts and cirrhosis. However, its association with pericarditis has not been investigated. Here, we describe a case of a female with PBC who presented with severe pericardial disease.

Clinical Case: A 57-year-old female with past medical history of primary biliary cirrhosis, thrombocytosis and type 2 diabetes who presented with sudden onset sharp chest pain, worse with movement or breathing. Workup was notable for elevated D-dimer, CT chest with no evidence of pulmonary embolism but did reveal a moderate pericardial effusion, further confirmed on echocardiogram. Nuclear stress test was unremarkable. CRP 13 mg/dL and ESR 82 mm/hr. Given concern for pericarditis, she was started on colchicine and ibuprofen. On further evaluation in clinic, cardiac MRI showed severe LGE and edema consistent with active pericarditis (figure 1) with mild constrictive physiology and worsening inflammatory markers (ESR 95 mm/hr, us-CRP 118mg/L). Therefore, decision was made to start triple therapy with the addition of prednisone. Repeat cardiac MRI 6 months later showed resolution of LGE and pericardial edema (figure 2). Given clinical improvement, patient was tapered off steroids, started anakinra and weaned off ibuprofen and colchicine. She remained on anakinra for two years after which she was transitioned to single therapy with colchicine with no episodes of recurrence.

Discussion: PBC commonly exists with liver autoimmune disorders, but its association with pericardial disease has not been investigated. NSAIDs are often used as first-line therapy for pericarditis, but can be detrimental in advanced liver failure, often seen in patients with PBC. Steroid use is not only advantageous in pericardial disease but also in PBC. As a result, it is critical to identify the link between pericardial disease and PBC to determine appropriate therapeutic management.