Evaluation and Management of Pericarditis in Rheumatic Diseases

Abstract

This review summarizes the evaluation for underlying rheumatic conditions in patients presenting with acute pericarditis, treatment considerations for specific rheumatic conditions, and the role of imaging in diagnosis and monitoring. Pericarditis may be one of the initial presentations of a rheumatic disease or identified in a patient with known rheumatic disease. There is also growing evidence for using anti-inflammatory and immunosuppressive agents for treating recurrent pericarditis, which can overlap with the treatment of rheumatic diseases.

Figure 1.. Flowchart on the diagnosis and management of pericarditis in rheumatic diseases

A flowchart demonstrating the diagnosis and management of rheumatic disease-associated pericarditis. ECG, electrocardiogram; CRP, C-reactive protein; cMRI, cardiac magnetic resonance imaging; NSAIDs, nonsteroidal anti-inflammatory drugs.

Figure 2.. Serial echocardiograms in a patient with SLE

A. Initial TTE with evidence of a large circumferential pericardial effusion with diastolic inversion of the right ventricular free wall (yellow arrow), right atrial inversion (red arrow), and excessive respiratory variation in the mitral valve assessed by Doppler (arrowhead). B. TTE showing recurrence of large pericardial effusion during early treatment with mycophenolate mofetil and steroids, with evidence of late right ventricular diastolic inversion (yellow arrow), right atrial inversion (red arrow) and excessive respiratory variation in the tricuspid valve assessed by Doppler (arrowhead). C. TTE showing resolution of pericardial effusion following treatment with rituximab. SLE, systemic lupus erythematosus, TTE, transthoracic echocardiogram

Figure 3.. Monitoring response to therapy using CMR in a patient with SLE pericarditis

A. Initial CMR images after treatment with colchicine showing thickened pericardium (red arrow) on T1 weighted images (I), pericardial LGE suggestive of active inflammation (II & III, yellow arrow), and an edematous pericardium on black blood T2-weighted images (IV, arrowhead). B. Follow-up CMR images after treatment with belimumab showing persistent pericardial thickening (I, red arrow), pericardial inflammation (II & III, yellow arrow), and edema (IV, arrowhead). C. Follow-up CMR images after treatment with IVIG showing improvement in pericardial thickening (I, red arrow) and pericardial fibrosis and inflammation on LGE sequences (II & III, yellow arrow) and axial T2 fat suppress dark blood sequences (IV, arrowhead). D. CMR images after treatment with rilonacept showing marked improvement in pericardial thickness (I, red arrow), minimal residual pericardial LGE (II & III, yellow arrow), with resolution of edema on black blood T2 weighted images (IV, arrowhead). CMR, cardiac magnetic resonance; SLE, systemic lupus erythematosus; LGE, late-gadolinium enhancement; IVIG, intravenous immunoglobulin