Diagnosis and management of myocardial involvement in systemic immune-mediated diseases: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Disease

Extract

Introduction

Systemic immune-mediated diseases (SIDs) include autoimmune and autoinflammatory diseases (AD) affecting at least two-organ systems.¹ Autoinflammatory diseases refer to a growing family of conditions characterised by episodes of unprovoked inflammation in the absence of high autoantibody titres or auto reactive T lymphocytes, reflecting a primary innate immune system dysfunction.¹ Conversely, autoimmune diseases are characterised by aberrant B, T and dendritic cell responses, leading to a break in tolerance against self-antigens, with predominantly cell-mediated or autoantibody-mediated responses in genetically susceptible individuals.^2–11 Autoantibodies (AAbs), when detectable, can promote inflammatory responses via immune complex formation and may directly affect target organ function,¹⁰ e.g. resulting, in cardiac autoimmunity, in electrical disturbance, cardiomyocyte dysfunction or loss and heart failure.^12–15 However, a dichotomous classification does not reflect clinical evidence and a continuum from purely autoinflammatory to purely autoimmune diseases should be considered (Figure  1).¹

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Cardiac involvement in SIDs is associated with adverse outcomes.^16–18 Currently there is a lack of up to date cardiological diagnostic workup in the scientific literature and in clinical practice, leading to poor scientific knowledge, late recognition or under diagnosis and under treatment of cardiac involvement.^16–18 Specific limitations include the use of scores only based on clinical findings (e.g. heart failure symptoms) to stratify patients with cardiac involvement as well as limited information based on state-of-the art non-invasive and invasive methodology.^16–18 Although all heart structures may be affected (see Supplementary material online, Table S1), we will focus on inflammatory and degenerative myocardial diseases, which may include: (i) myocarditis evolving to a dilated cardiomyopathy (DCM) or a hypokinetic non-dilated cardiomyopathy; (ii) endomyocarditis and endomyocardial fibrosis.^19–22 The aim of this multidisciplinary position paper by the European Society of Cardiology (ESC) Working Group on Myocardial and Pericardial Disease is to help cardiologists and non-cardiac specialists to select the appropriate diagnostic workup in SIDs, setting the stage for future therapeutic choices.^{11  ,  23–27}