Immunologic Aspects of Neurological and Neuromuscular Diseases

Zweiman, Burton

doi:10.1001/jama.1987.03400200176022

Cited by 6 publications

(1 citation statement)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…AHA which gave a broad striational pattern on longitudinal sections of heart and skeletal muscle were classified as "cross-reactive 2". This pattern had been previously found in 30 -40% of sera from myasthenia gravis patients without thymoma and in all myasthenic patients with thymoma [63]. These striational antibodies have been shown to react with the A band of the myofibrils of striated muscle, and to cross-react with thymus myoepithelial cells.…”

Section: Dilated Cardiomyopathy (Dcm) and Myocarditis: Classificationsupporting

confidence: 64%

Clinical implications of anti-heart autoantibodies in myocarditis and dilated cardiomyopathy

et al. 2008

View full text Add to dashboard Cite

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.

show abstract

Section: Dilated Cardiomyopathy (Dcm) and Myocarditis: Classificationsupporting

confidence: 64%