Potassium Channel Block and Novel Autoimmune-Associated Long QT Syndrome.
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.
Research and Development Service, VA New York Harbor Healthcare System, 800 Poly Place, Brooklyn, NY 11209, USA; Departments of Medicine, Cell Biology and Pharmacology, SUNY Downstate Medical Center, 450 Clarkson Avenue, Brooklyn, NY 11203, USA; Department of Medicine, NYU School of Medicine, 550, 1st Avenue, New York, NY 10016, USA.
Mohamed
M
Boutjdir
Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Policlinico "Le Scotte", Viale Bracci, Siena 53100, Italy.
Pietro Enea
PE
Lazzerini
Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Policlinico "Le Scotte", Viale Bracci, Siena 53100, Italy.
Pier Leopoldo
PL
Capecchi
Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Policlinico "Le Scotte", Viale Bracci, Siena 53100, Italy.
Franco
F
Laghi-Pasini
Research and Development Service, VA New York Harbor Healthcare System, 800 Poly Place, Brooklyn, NY 11209, USA; Departments of Medicine, Cell Biology and Pharmacology, SUNY Downstate Medical Center, 450 Clarkson Avenue, Brooklyn, NY 11203, USA. Electronic address: nelsherif@aol.com.
Nabil
N
El-Sherif
D001323:Autoantibodies; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D008133:Long QT Syndrome / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D015221:Potassium Channels
D001323:Autoantibodies; D015221:Potassium Channels
Anti-SSA/Ro antibodies; Autoimmune disease; Cardiac arrhythmia; Long QT syndrome; Potassium channels