Intractable Recurrent Pericarditis: Rest, Medication, or Surgery?

A 48-year-old female lawyer from South Africa presents for an opinion regarding her management following a complicated course of intractable recurrent pericarditis. Her medical history also includes mitral valve prolapse with mild mitral regurgitation, steroid-induced glaucoma, cataracts, gastritis, osteoporosis, and kidney stones. The patient is an athlete and exercises four to five times weekly.

She was first diagnosed with pericarditis two years ago. She had a delayed diagnosis after a 3-month prodrome of diarrhea, cough and dyspnea with elevated Coxsackie B antibody titers. One month later, she developed typical pleuritic chest pain, high grade fever, and a small pericardial effusion on computed tomography of the chest. She was diagnosed with viral pericarditis and started first on diclofenac 75 mg orally twice daily, and later on prednisone 30 mg once daily and colchicine 0.6 mg once daily. Cardiac magnetic resonance imaging of the heart showed increased pericardial signal on delayed enhancement sequences and an increased pericardial thickness confirming active pericarditis. She underwent endomyocardial biopsies due to persistent disease, which showed active inflammation consistent with myopericarditis, without evidence of any specific secondary causes (Figure 1).

Figure 1

Figure 1
Figure 1: (A) Electrocardiogram with subtle ST elevation and PR-depression confined to the inferior limb leads. (B) longitudinal 2D speckle tracking Bull's eye plot, demonstrating abnormal strain values in the basal infero-lateral segment. (C) Short axis CMR T2-STIR imaging showing increased pericardial (arrow) signals within the anterior and anteroseptal segments. (D) Short axis CMR delayed sequences after injection of gadolinium contrast agent showing increased myocardial (*) and pericardial (arrow) signals within the mid-inferior and inferolateral segments. This constellation of findings is consistent with peri-myocarditis.

The initial episode resolved, however, she had multiple recurrences on resumption of her exercise program with incessant pericarditis despite aggressive therapy with more than ten emergency room presentations and hospitalizations. Because of her inability to wean down steroids, she was tried on multiple immunosuppressive medications: intravenous immune globulin, intrapericardial instillation of corticosteroids, azathioprine, methotrexate, and finally, anakinra. With these treatments she developed significant adverse effects including fungal and bacterial esophagitis (methotrexate), Stevens-Johnson syndrome (anakinra), and steroid-induced glaucoma, cataracts, and osteoporosis.

Eight years after her initial episode, she was still having recurrent bouts of pericarditis despite restriction from exercise and medication regimen of methylprednisolone 6 mg daily, meloxicam 15 mg daily and aspirin 250 daily. She ultimately decided to undergo radical pericardiectomy at a center with specific expertise in this surgery. The entire pericardium was removed to the level of the oblique sinus and the patient's symptoms resolved until this day.

Which of the following is true regarding the treatment approach for patients with recurrent pericarditis?

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