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Specific heart muscle disorders 

Specific heart muscle disorders
Chapter:
Specific heart muscle disorders
Author(s):

Oliver P. Guttmann

, and Perry Elliott

DOI:
10.1093/med/9780198746690.003.0355
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date: 05 March 2021

Systemic immune-mediated diseases are autoimmune and autoinflammatory diseases affecting at least two-organ systems. Autoinflammatory diseases are a family of conditions characterized by episodes of unprovoked inflammation in the absence of high autoantibody titres or autoreactive T lymphocytes, reflecting a primary dysfunction of the innate immune system. Autoimmune diseases are characterized by aberrant B, T, and dendritic cell responses with predominantly cell-mediated or autoantibody-mediated responses to self-antigens in genetically susceptible individuals. Cardiovascular involvement is systemic immune-mediated diseases may be occult and often goes undetected, but is associated with a poor prognosis. As any anatomical structure in the heart may be involved, patients can present with one or more features consistent with pericarditis, myocarditis, endocarditis, and vasculitis. There is often no correlation between the extent of systemic disease and cardiac involvement.

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