Recurrent Pericarditis with Autoinflammatory Features
A 53-year-old woman presents with a two-year history of recurrent fever, shortness of breath and an erythematous rash located primarily on the face, neck and upper chest. She also complains of frequent pleuritic chest pain. Review of systems is positive for fatigue, weight gain, joint pain, myalgia, dry eyes and dry mouth, nausea and abdominal pain. The patient denies orthopnea, paroxysmal nocturnal dyspnea, peripheral edema, palpitations or dizziness. Her blood pressure is 138/90 mmHg, temperature is 98.4F, respiratory rate is 14 breaths per minute, with an oxygen saturation of 95%, and heart rate at 72 beats per minute. Physical exam is notable for an obese woman with erythematous patches and plaques on face, chest and trunk. Cardiopulmonary exam is significant for elevated jugular venous pressure at 9 cm H2O, pericardial friction rub and reduced breath sound in the lung bases. The remainder of the exam is within normal limits. A complete blood count is unremarkable. B-type Natriuretic Peptide (BNP) is 271 pg/mL. Westergren sedimentation rate (WSR) and C-reactive protein (CRP) levels are elevated at 39 mm/hour and 9.8 mg/dl respectively. Chest X-Ray reveals small bilateral pleural effusions and an enlarged cardiac silhouette. Transthoracic echocardiography is notable for pericardial enhancement anteriorly adjacent to the right ventricle and a small pericardial effusion. Estimated ejection fraction (EF) noted to be at 60%. Cardiac MRI performed is shown below (Fig A and B).
Figures A & B
Which of the following is the most likely diagnosis in this patient?