Latest Treatment Approach for Recurrent Lupus Pericarditis

42 year-old African-American female from Sudan with history of systemic lupus erythematous (SLE) of 9 years, diffuse interstitial lung disease of 3 years, and Sjogren's syndrome who presented to cardiology clinic for evaluation of pericardial effusion. 10 days prior to presentation, she developed chest pain and shortness of breath treated by her primary care physician (PCP) as "lung infection" with clarithromycin. The chest pain was described as substernal, mild (5 out of 10), intermittent, crushing, which worsened with deep inspiration or lying flat and radiated to the left shoulder. Her dyspnea worsened with minimal exertion, lying down, and she required 2-3 pillows to sleep. Due to the persistence of symptoms, her PCP ordered a chest computed tomography (CT) with contrast which revealed a large circumferential pericardial effusion (23 mm). Her medication regimen at this time included hydroxychloroquine 200mg oral twice daily, mycophenolate mofetil (MMF) 1000mg oral twice daily, low dose aspirin, trimethoprim/sulfamethoxazole 80 mg-400 mg oral daily, budesonide, and formoterol. Of note, she did not tolerate azathioprine in the past due to nausea and vomiting. Her family history was negative for autoimmune disease and denied any history of smoking or chemical exposure.

Upon presentation, her blood pressure was 100/64 mmHg, respiratory rate of 16 per minute, pulse of 84 beats per minute, temperature 36.6C and BMI of 34.3 kg/m2. Cardiovascular examination was notable for normal S1 and S2, no murmur or rubs, jugular venous pressure of 12 cm H20 with inspiratory collapse and, no pedal edema. Lungs auscultation revealed diffuse bilateral rales. Her abdomen was soft, non-tender, with normal bowel sounds. Complete blood count showed hemoglobin of 10.8 g/dl (normal: 11.5-15.5g/dL) with normal white blood count and platelets. Comprehensive metabolic panel, troponin T, and creatine kinase levels were within normal limits. C-reactive protein (CRP) level was 5.5 mg/dl (normal: 0.0-1 mg/dL), Westergren sedimentation rate (WSR) was 76 mm/hr (normal: 0-20 mm/hr) and anti-DNA assay was 660 IU/mL (normal: <30 IU/mL). Electrocardiogram showed normal sinus rhythm at 84 beats per minute without PR depression or ST elevation. Her echocardiogram showed a left ventricular ejection fraction of 64%, a moderate, circumferential pericardial effusion, septal bounce, and dilation of inferior vena cava at 20 mm with minimal collapse during inspiration (Figure 1; Video 1).

Figure 1: Transthoracic echocardiogram: parasternal short axis view (prior to pericardial window)

Figure 1

Video 1: Apical four chamber view on transthoracic echocardiography

Given the persistence of symptoms, significant pericardial effusion size, and for diagnostic purposes, she was referred for elective pericardial window and biopsy. 600 ml of bloody fluid was drained with improvement in her symptoms and the biopsy results revealed moderate fibrosis and chronic inflammation without any evidence of malignancy. Additional studies were remarkable for negative coxsackie antibody titers, tuberculin skin test was non-specific, likely as she received Bacillus Calmette-Guerin in the past, and her lyme antibody screen was negative.

Following the pericardial window, her dyspnea and exercise capacity have improved but she is still experiencing chest pain. She underwent a cardiac magnetic resonance imaging (MRI) which showed evidence of abnormal diffuse delayed gadolinium enhancement of the visceral and parietal pericardium consistent with moderate pericardial inflammation (Figure 2 & 3; Video 2). CRP level is 2.6 mg/dl and WSR level is 53 mm/hr.

Figure 2: Cardiac MRI delayed gadolinium-enhancement analysis - short axis view

Figure 2

Figure 3: Cardiac MRI delayed gadolinium-enhancement analysis - three chamber view

Figure 3

Video 2: Four chamber view on cardiac magnetic resonance imaging

Which of the following is the next best step in management?

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