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Autoimmun Rev. 2006 May;5(5):306-13. Epub 2005 Dec 9.

Connective tissue diseases and cardiac rhythm disorders: an overview.

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Department of Clinical Medicine and Immunological Sciences, Division of Clinical Immunology, University of Siena, Viale Bracci, 53100 Siena, Italy.


Cardiovascular involvement is common in connective tissue diseases (CTD), with relevant implications in terms of morbidity and mortality. Rhythm disturbances, i.e. conduction defects and tachyarrhythmias, represent a frequent clinical manifestation of CTD-associated cardiovascular damage and a possible cause of sudden death. The underlying arrhythmogenic mechanisms are probably multiple and intriguing, even though the myocardial fibrosis frequently observed at the pathological examination seems to play a pivotal role. Myocardial fibrosis is produced directly by inflammatory processes, or indirectly as a consequence of coronary artery occlusive disease, and it may affect the conduction system also representing the pathological substrate for reentry circles. An overview of CTD-associated cardiac rhythm disturbances is here provided. Among CTD-associated rhythm disorders, congenital heart block (CHB), which represents the main feature of neonatal lupus, a rare syndrome related to the transplacental passage of autoantibodies from anti-Ro/SSA-positive mother to their newborns, seems to acknowledge a peculiar mechanism of disease possibly dependent on a direct arrhythmogenicity of anti-Ro/SSA antibodies. Moreover, new anti-Ro/SSA-associated EKG abnormalities have been recently described in children (sinus bradycardia and corrected QT (QTc) interval prolongation) as well as in adults (QTc interval prolongation).

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