autoimmune-cardiomyopathy-a-review-and-pooled-analysis-of-pathophysiology-diagnosis-and-clinical-management

Abstract

Dilated cardiomyopathy (DCM), characterized by dilatation of cardiac chambers and systolic dysfunction, is one of the leading causes of severe heart failure (HF) and the most common indication for heart transplantation. Despite significant therapeutic improvements, its incidence and mortality is persistently high largely attributable to heterogeneous aetiology. Infectious diseases and genetic factors predominate research on the causes of DCM. However, evidence over the past two decades reveal distinct autoantibodies or other immune factors in heterogeneous subsets of DCM suggesting the involvement of autoimmune mechanisms in the pathogenesis of DCM. Hitherto, accumulated evidence demonstrates abnormalities in cell-mediated immunity such as altered lymphocyte function and relative proportion of lymphocytes subsets, activated cytokine system, inappropriate expression of histocompatibility complex on cardiac tissue and the expression of adhesion molecules on cardiomyocytes. This evidence suggest a subset of DCM patients is autoimmunity-mediated. This review aims to summarize the contemporary understanding of the characteristics of autoimmune cardiomyopathy in humans, proposed pathophysiology mechanisms as well as the ongoing clinical investigations that have focused on autoimmunity as a target for therapy.