Fatal Myopericarditis in a Patient With Lupus Erythematosus Supported by Extracorporeal Membrane Oxygenation: A Case Report

Abstract

Systemic lupus erythematosus (SLE) may occur in any organ. In patients with SLE, myocarditis is extremely rare and potentially life-threatening. Herein, we report on a patient with lupus myocarditis, diagnosed by myocardial biopsy, immunologic tests, and clinical manifestations. Our findings suggest that securing time for diagnosis via extracorporeal membrane oxygenation and other intensive care is helpful for obtaining a good prognosis.

Keywords: Extracorporeal membrane oxygenation; Myocarditis; Systemic lupus erythematosus.

Figure 1

Chest X-rays of the patients over time. (A) At the time of previous hospital admission, 17 days before visit; (B) 1st day of hospitalization; (C) 3rd day of hospitalization (endomyo-cardial biopsy was performed); (D) 12th day of hospitalization (2nd pericardiocentesis was performed due to aggravated pericardial effusion); (E) 21st day of hospitalization (9 days after the restart of steroid therapy); (F) 49th day of hospitalization, at the time of discharge.

Figure 2

Echocardiogram. Still images (A~C) have been captured after extracorporeal membrane oxygenation and before pericardiocentesis. (A) End diastole of the parasternal long-axis view shows a thickened LV wall with decreased LV cavity; (B) End diastole of the modified four-chamber view. Pericardial effusion compressing the right ventricular lateral wall is seen (arrows in A, B); (C) Mitral inflow pattern; the images (D~F) have been captured 2 weeks after the steroid therapy. (D~F) The ventricular wall thickness returned to the normal range compared with the initial echocardiogram (A). Mitral valvular calcification, thickening, doming, and elevated mean trans-mitral pressure gradient of 5 mmHg suggested concomitant mild mitral stenosis. Ao: ascending aorta, IVS: interventricular septum, LA: left atrium, LV: left ventricular cavity, MV: mitral valve, PW: posterior wall, RV: right ventricle, RA: right atrium.

Figure 3

Pathologic findings. (A) Scattered lymphocytes and loss of myocardium replaced by progressive fibrosis (arrows) (H&E stain, ×11); (B) Immuno-histochemistry of CD3. The diffusely scattered lymphocytes have been stained dominantly with CD3 (×11).

Figure 4

Changes in laboratory findings and treatment according to hospitalization and outpatient follow-up periods. EMB: endomyocardial biopsy, ECMO: extracorporeal membrane oxygenation, MPD: Methylprednisolone, HCQ: hydroxychloroquine, WBC: white blood cell, CRP: C-reactive protein.