Endomyocardial biopsies in the diagnosis of myocardial involvement in systemic lupus erythematosus

Abstract

Background Endomyocardial biopsy (EMB) is considered the gold standard for diagnosing myocardial involvement in most inflammatory conditions, including systemic lupus erythematosus (SLE). However, EMBs are rarely performed, and most of the myocardial histopathology reports in SLE consist of postmortem data. We therefore sought to describe the histopathologic findings of contemporary EMBs in SLE performed in clinical practice. Methods A retrospective review of histopathology reports from SLE patients who underwent EMB from 1994 to 2017 was performed. A total of 41 SLE patients had cardiac pathology reports. Of these, 11 histopathology reports were EMBs, and the remaining were valvular specimens. Results A total of 11 SLE EMBs were reviewed. It was found that 45% of the patients had hypertension, 27% had coronary artery disease, 9% had hyperlipidemia, and 36% had end-stage renal disease. None had diabetes or smoked. The mean left ventricular ejection fraction was 37%. On histopathology, 10 had mild interstitial fibrosis, 9 had myocyte hypertrophy, 3 had organized blood clots, and 3 had a mild infiltration of lymphocytes and macrophages without clear evidence of myocarditis. None had vasculitis, endocarditis, ischemia, amyloid deposition, or lamellar or curvilinear inclusions. Conclusion EMBs are rarely performed in SLE. In this case series, nonspecific interstitial fibrosis and myocyte hypertrophy were the most common findings, suggesting EMBs have limited value in the diagnosis of cardiac involvement in SLE and rather rule out competing conditions. These data support the need for diagnostic methods with high sensitivity and specificity for SLE heart disease.

Predictive Value of Echocardiographic Strain for Myocardial Fibrosis and Adverse Outcomes in Autoimmune Diseases

“…Second, the etiological heterogeneity of the study population confers some limitations to our research. Nevertheless, myocardial fibrosis is a common feature on cardiac pathology in patients with SLE ( 6 ), IIM ( 40 ), SS ( 41 ), and SSc ( 2 ). The shared pathophysiologic mechanisms of myocardial involvement in AIDs, such as immune complex deposition and chronic cumulative inflammation, may account for the commonality across imaging findings.…”

“…Indeed, histopathological studies have demonstrated that myocardial fibrosis is prevalent in AIDs and indicates impaired cardiac function ( 2 , 5 ). For instance, a high frequency (90%) of interstitial fibrosis in myocardium occurred in patients with systemic lupus erythematosus (SLE) based on endomyocardial biopsy ( 6 ). Although endomyocardial biopsy is the gold standard for the diagnosis of myocardial involvement, its invasive properties and complications sometimes limit its clinical application.…”

Diffuse myometrium thinning and placenta accreta spectrum in a patient with systemic lupus erythematosus (SLE): a case report and review of the literature

“…We could also appreciate a microscopical observation of fibrosis and vasculitis at the myometrium. Interstitial fibrosis to myocardial involvement [ 15 ] and gynecological vasculitis in SLE have been reported to date [ 16 ], causing activating the complement cascade to damage the microvascular structure of the uterus. In particular, our patient had repeated paralytic ileus and lupus enteritis without steroid treatment for one year.…”

A Unique Case of Systemic Lupus Erythematosus Myocarditis Complicated by Plasmapheresis-Responsive Cardiogenic Shock

“…This patient had 2 separate endomyocardial biopsies: neither of which revealed the classic inflammatory infiltrate in association with myocyte degeneration ( 3 ). In the most contemporary published series of endomyocardial biopsies of serology-proven lupus patients with decreased LVEF, none of the relevant samples had features characteristic for lupus myocarditis.…”

“…In the most contemporary published series of endomyocardial biopsies of serology-proven lupus patients with decreased LVEF, none of the relevant samples had features characteristic for lupus myocarditis. Findings included interstitial fibrosis, myocyte hypertrophy, and mild lymphocytic and macrophage infiltration without clear evidence of myocarditis ( 3 ). Therefore, there is growing evidence that CMR imaging with late gadolinium enhancement may be a better diagnostic modality for lupus myocarditis ( 4 ).…”