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This article reviews advances in the pathogenesis of anti-SSA/Ro antibody-induced corrected QT (QTc) prolongation in patients with autoimmune diseases; particularly connective tissue disease (CTD). Evidence shows that anti-SSA/Ro antibody-positive patients with CTD show QTc prolongation and complex ventricular arrhythmias. Molecular and functional data provide evidence that the human ether-a-go-go-related gene potassium channel conducting the rapidly activating delayed rectifier potassium current is directly inhibited by anti-SSA/Ro antibodies, resulting in action potential duration prolongation leading to QT interval lengthening. Routine electrocardiogram screening in anti-SSA/Ro antibody-positive patients and counseling for patients with other QTc prolonging risk factors is recommended.

Boutjdir, M., Lazzerini, P.E., Capecchi, P.L., LAGHI PASINI, F., El Sherif, N. (2016). Potassium Channel Block and Novel Autoimmune-Associated Long QT Syndrome. CARDIAC ELECTROPHYSIOLOGY CLINICS, 8(2), 373-384 [10.1016/j.ccep.2016.02.002].

Potassium Channel Block and Novel Autoimmune-Associated Long QT Syndrome

LAZZERINI, PIETRO ENEA;CAPECCHI, PIER LEOPOLDO;LAGHI PASINI, FRANCO;
2016-01-01

Abstract

This article reviews advances in the pathogenesis of anti-SSA/Ro antibody-induced corrected QT (QTc) prolongation in patients with autoimmune diseases; particularly connective tissue disease (CTD). Evidence shows that anti-SSA/Ro antibody-positive patients with CTD show QTc prolongation and complex ventricular arrhythmias. Molecular and functional data provide evidence that the human ether-a-go-go-related gene potassium channel conducting the rapidly activating delayed rectifier potassium current is directly inhibited by anti-SSA/Ro antibodies, resulting in action potential duration prolongation leading to QT interval lengthening. Routine electrocardiogram screening in anti-SSA/Ro antibody-positive patients and counseling for patients with other QTc prolonging risk factors is recommended.
2016
CARDIAC ELECTROPHYSIOLOGY CLINICS
Boutjdir, M., Lazzerini, P.E., Capecchi, P.L., LAGHI PASINI, F., El Sherif, N. (2016). Potassium Channel Block and Novel Autoimmune-Associated Long QT Syndrome. CARDIAC ELECTROPHYSIOLOGY CLINICS, 8(2), 373-384 [10.1016/j.ccep.2016.02.002].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11365/1000011
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