Recurrent Pericarditis: A Disease of All Ages
An 11-year-old male presents to the office for an evaluation regarding recurrent pericarditis. He has left-sided chest pain, pleuritic, relieved on bending forward, worsened on laying down, and irradiates to both shoulders. He denies shortness of breath on exertion, orthopnea, significant weight gain, leg swelling or history of syncope.
In the last four years, he has had approximately 25 flares of chest pain. He reports two different types of chest pain: sharp, stabbing pain when he suspects there is no fluid around his heart and a "foggy, hard to breathe" feeling when he suspects there is fluid around his heart. His mother reports five to seven emergency department visits for chest pain, two of which included overnight admissions. His autoimmune serology workup was negative, including antinuclear and smooth muscle antibodies, rheumatoid factor, and antineutrophil cytoplasmic antibodies (ANCA). He has been treated initially with ibuprofen and colchicine, then prednisone and canakinumab were added because of inadequate response to ibuprofen. His family history is relevant for systemic lupus erythematosus (SLE) in his paternal aunt and rheumatoid arthritis in his paternal great uncle.
At the office, his blood pressure was 113/69 mmHg, his pulse was 70 beats per minute, and his oxygen saturation was 98% on room air. On exam, his lungs were clear, and he had irregular S1 and S2 with no murmurs or pericardial knock, no jugular venous distention, no Kussmaul's sign, and no peripheral edema.
His ultrasensitive C-reactive protein was 0.5 mg/deciliter (normal less than 3.1 mg/dL), and erythrocyte sedimentation rate was 2 mm/hour (normal less than 15 mm/hour). Electrocardiogram showed sinus arrhythmia. Transthoracic echocardiography showed an ejection fraction of 62% without any pericardial effusion or constriction (Figure 1). Cardiac magnetic resonance imaging (MRI) revealed diffuse pericardial delayed enhancement, most prominent inferiorly and posteriorly, along with increased pericardial signal intensity on T2 short-tau inversion recovery (STIR) imaging (Figure 2). There was no pericardial effusion, pericardial thickening, or features of concomitant constrictive pericarditis.
He was switched to anakinra (since canakinumab was deemed ineffective), colchicine, prednisone and meloxicam. He had a right forearm biopsy of a skin nodule which showed histologic features of middle vessel vasculitis suggestive of cutaneous polyarteritis nodosa.
Despite quadruple anti-inflammatory therapy, his pericarditis symptoms remained very debilitating; he is now unable to play baseball and has difficulty ambulating around the house. The decision was made to undergo a radical pericardiectomy. Pathology of the excised pericardium exhibited diffuse fibrous adhesions with minimal chronic inflammation. After the surgery, his chest pain resolved; however, he continued to have left-sided abdominal pain, diarrhea, and elevated liver enzymes. Magnetic resonance enterography showed wall thickening and mucosal enhancement of the rectum and sigmoid colon that may be secondary to inflammatory colitis.
Which of the following is true regarding this child's early-onset pericarditis?