Treating Sarcoidosis to Treat Underlying Recurrent Pericarditis

A 58-year-old male presented to clinic for management of recurrent pericarditis. Two years ago, he underwent aortic valve replacement with size 25 Carpentier-Edwards valve. Pre-existing medical problems include heart failure with reduced ejection fraction, supraventricular tachycardia, hyperlipidemia, and hypertension. Prior to this visit, the patient had four episodes of pericarditis within a year which were treated with ibuprofen and steroid taper. His echocardiogram showed moderate concentric left ventricular hypertrophy and moderate pericardial effusion. During these recurrences, he was treated with a short course of colchicine and tapering dose of steroids over 2 weeks.

At this point, he presented to the clinic with episodic chest pain, exertional dyspnea, and a weight gain of 10 pounds over 1-year period. His blood pressure was 118/73 mmHg, pulse rate was 70 beats per minute and oxygen saturation 97% on room air. On auscultation his lungs were clear and cardiac auscultation was unremarkable for any rub or murmurs. No jugular venous distension or Kussmaul's sign was detected. In the lower extremities, 1+ pitting edema bilaterally was noticed. Laboratory investigations revealed ultra-sensitive C-reactive protein (us-CRP) level of 1.6 mg/L (normal less than 3.1 mg/L) and angiotensin converting enzyme (ACE) of 52 U/L (normal less than 49 U/L). His transthoracic echocardiogram showed an ejection fraction of 35%. His cardiac magnetic resonance imaging (CMR) (Image 1) demonstrated areas of pericardial prominence with maximal thickness of 3 mm along the free wall of right ventricle (red arrow), mild pericardial delayed enhancement along the free wall of the entire cardiac base and mid septum, prominent nodules in the right upper lung lobe (blue arrow) and significant mediastinal adenopathy, which were concerning for sarcoidosis.

Patient was referred to pulmonology and a cardiac positron emission tomography (cPET) scan was performed. The cPET scan (Image 2) showed areas of enhanced uptake in the basal anteroseptal and basal inferolateral myocardial segments concerning for myocardial inflammation and hence, a diagnosis of cardiac sarcoidosis along with pericarditis was established. Disease modifying anti-rheumatic drugs (leflunomide and methotrexate) were prescribed. For management of recurrent pericarditis, prednisone 40 mg, ibuprofen 800 mg thrice daily and colchicine 0.6 mg twice daily. Patient reported improvement and did not have further recurrence of pericarditis.

Image 1

Figure 1
Image 1: Red arrow: Pericardial prominence noted along the ventricular wall seen on CMR. Blue arrow: Lung nodules observed on CMR.

Image 2

Figure 2
Image 2: Area of enhanced myocardial uptake seen on cPET.

What is the next best step in preventing future recurrences?

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