Defining the Underlying Cause of Pericarditis

A 59 year-old male presents to cardiology clinic for exacerbations of recurrent chest pain. His first episode occurred a year ago when he noticed a fever of 102.7F, palpitations and pleuritic chest pain. He had no prior medical conditions and no recreational drug use. Family history was notable for a sister with lupus and mother with Behcet's syndrome although he lacked rheumatologic symptoms or signs on exam. His CRP was 15.6mg/L (<3mg/L) and ESR was 12mm/hr (0-15mm/hr). A left heart catherization was performed and showed nonobstructive coronary artery disease. He then had a cardiac magnetic resonance imaging (MRI/CMR) that showed mildly thickened pericardium with late gadolinium enhancement of the pericardium without pericardial effusion or abnormal ventricular septal motion (Figure 1C). He was diagnosed with acute pericarditis. He was discharged on colchicine and ibuprofen for 6 months.

Unfortunately, 10 days after stopping this regimen after his 6 months course, he developed another episode of chest pain again with fever and dyspnea. His original dose of colchicine was doubled, and ibuprofen was restarted. His symptoms gradually resolved on this dual anti-inflammatory regimen.

He then had his third episode of angina two months later despite still being on therapy. In the emergency department, they diagnosed him with pericardial tamponade and by cardiothoracic surgery performed a pericardial window. Both the pericardial fluid cultures and the tissue culture grew Cutibacterium acnes, formerly known as Propionibacterium acnes. Inflammatory markers were elevated with CRP 138.8mg/L and ESR of 9 mm/hr. Infectious disease was consulted and recommended a two-week course of intravenous ceftriaxone. After which, he transitioned to doxycycline, however, he had recurrence of his chest pain that was resolved with increasing the antibiotic dosage. During this time, he had an outpatient rheumatology visit where they noted a history of dry eyes and chronic hip pain attributed to leg braces as a child. Their workup was significant for ANA titer of 1:160 with speckling as well as a positive anti-SSA titers of 166. Antibodies for SSB, cyclic citrullinated peptide, double stranded-DNA, Smith, ribonucleoprotein were unremarkable as were complement levels and serum protein electrophoresis. Given his family history of lupus and Behcet's syndrome along with these titers, he was offered hydroxychloroquine but declined.

Ten months after his initial episode, he had his fourth pericarditis flare. His CRP was 293mg/L and ESR was 33 mm/hr. Transthoracic echocardiogram revealed a small pericardial effusion and his regimen of colchicine and ibuprofen was continued.

He then presents to the pericardial clinic a month after his most recent exacerbation where he noted intermittent chest pain with radiation to the shoulders that was worse when lying down and exertion. He was on colchicine 1.2mg daily, indomethacin 500mg twice a day, doxycycline 100mg twice a day and atorvastatin 20mg daily. Physical exam was unrevealing. Laboratory studies at this visit showed normal inflammatory markers with CRP 0.8 mg/L and ESR of 2mm/hr along with unremarkable rheumatology workup including ANA, Rheumatoid Factor and IL-6. Electrocardiogram (ECG) showed normal sinus rhythm with left ventricular hypertrophy (Figure 1A). Chest X-ray was unremarkable. Transthoracic echocardiogram revealed normal diastolic function based on mitral inflow and tissue Doppler and did not show pericardial effusion, annulus reversus or respirophasic shift (Figure 1B). Cardiac MRI showed improvement of mildly thickened pericardium with persistent late gadolinium enhancement (Figure 1D).

Figure 1
Figure 1: (A) ECG with normal sinus rhythm and left ventricular hypertrophy. (B) Doppler echo showing normal mitral inflow. (C) Short axis CMR with late gadolinium enhancement around the inferior and lateral walls during his initial flare. (D) Short axis CMR with late gadolinium enhancement around the pericardium 11 months later.

How would you treat this patient's chronic, recurrent pericarditis?

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