Document Detail

Clinical implications of anti-heart autoantibodies in myocarditis and dilated cardiomyopathy.
MedLine Citation:
PMID:  18176863     Owner:  NLM     Status:  MEDLINE    
Dilated cardiomyopathy (DCM), a leading cause of heart failure and heart transplantation in younger adults, is characterized by dilatation and impaired contraction of the left or both ventricles; it may be idiopathic, familial/genetic (20-30%), viral, and/or immune. On endomyocardial biopsy there is chronic inflammation in 30-40% of cases. Mutations in genes encoding myocyte structural proteins, cardiotoxic noxae and infectious agents are known causes; due to high aetiologic and genetic heterogeneity, the gene defects identified so far account for a tiny proportion of the familial cases. In at least two thirds of patients, DCM remains idiopathic. Myocarditis may be idiopathic, infectious or autoimmune and may heal or lead to DCM. Circulating heart-reactive autoantibodies are found in myocarditis/DCM patients and symptom-free relatives at higher frequency than in normal or noninflammatory heart disease control groups. These autoantibodies are directed against multiple antigens, some of which are expressed only in the heart (organ-specific); some autoantibodies have functional effects on cardiac myocytes in vitro as well as in animal models. Depletion of nonantigen-specific antibodies by extracorporeal immunoadsorption is associated with improved ventricular function and reduced cardiac symptoms in some DCM patients, suggesting that autoantibodies may also have a functional role in humans. Immunosuppression seems beneficial in patients who are virus-negative and cardiac autoantibody positive. Prospective family studies have shown that cardiac-specific autoantibodies are present in at least 60% of both familial and non familial pedigrees and predict DCM development among asymptomatic relatives, years before clinical and echocardiographic evidence of disease. Animal models have shown that autoimmune myocarditis/DCM can be induced by virus as well as reproduced by immunization with a well-characterized autoantigen, cardiac myosin. Thus, in a substantial proportion of patients, myocarditis and DCM represent different stages of an organ-specific autoimmune disease, that represents the final common pathogenetic pathway of infectious and noninfectious myocardial injuries in genetically predisposed individuals.
Alida L P Caforio; Francesco Tona; Stefania Bottaro; Annalisa Vinci; Greta Dequal; Luciano Daliento; Gaetano Thiene; Sabino Iliceto
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Autoimmunity     Volume:  41     ISSN:  1607-842X     ISO Abbreviation:  Autoimmunity     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-01-07     Completed Date:  2008-01-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8900070     Medline TA:  Autoimmunity     Country:  England    
Other Details:
Languages:  eng     Pagination:  35-45     Citation Subset:  IM    
Division of Cardiology, Department of Cardiological, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.
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MeSH Terms
Antibody Specificity
Autoantibodies / blood*,  immunology*
Autoantigens / immunology
Autoimmune Diseases* / immunology,  physiopathology
Cardiac Myosins / immunology*
Cardiomyopathy, Dilated* / immunology,  physiopathology
Myocarditis* / immunology,  physiopathology
Myocardium / immunology*
Reg. No./Substance:
0/Autoantibodies; 0/Autoantigens; EC 3.6.1.-/Cardiac Myosins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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