Potassium Channel Block and Novel Autoimmune-Associated Long QT Syndrome

Card Electrophysiol Clin. 2016 Jun;8(2):373-84. doi: 10.1016/j.ccep.2016.02.002. Epub 2016 Mar 26.

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.

Keywords: Anti-SSA/Ro antibodies; Autoimmune disease; Cardiac arrhythmia; Long QT syndrome; Potassium channels.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Autoantibodies
  • Autoimmune Diseases* / complications
  • Autoimmune Diseases* / immunology
  • Autoimmune Diseases* / physiopathology
  • Humans
  • Long QT Syndrome* / etiology
  • Long QT Syndrome* / immunology
  • Long QT Syndrome* / physiopathology
  • Potassium Channels*

Substances

  • Autoantibodies
  • Potassium Channels