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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

2017; Oxford University Press; Volume: 38; Issue: 35 Linguagem: Inglês

10.1093/eurheartj/ehx321

1522-9645

Alida L.P. Caforio, Yehuda Adler, Carlo Agostini, Yannick Allanore, Aris Anastasakis, Michael Arad, Michael Böhm, Philippe Charron, Perry Elliott, Urs Eriksson, Stephan B. Felix, Pablo García‐Pavía, É. Hachulla, Stéphane Heymans, Massimo Imazio, Karin Klingel, R Marcolongo, Marco Matucci‐Cerinic, Antonis Pantazis, Sven Plein, Valeria Poli, Angelos G. Rigopoulos, Petar Seferović, Yehuda Shoenfeld, José Luis Zamorano, Aleš Linhart,

Atherosclerosis and Cardiovascular Diseases

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