Anti-Ro/SSA antibodies and cardiac rhythm disturbances: Present and future perspectives

Santos-Pardo, Irene; Villuendas, Roger; Salvador-Corres, Iñaki; Martínez-Morillo, Melania; Olivé, Alejandro; Bayés‐Genís, Antoni

doi:10.1016/j.ijcard.2014.11.002

Cited by 16 publications

(15 citation statements)

References 55 publications

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“…Moreover, Ro-52kD antigen immunized guinea-pigs showed QTc prolongation on ECG after developing high titers of anti-Ro/SSA ( 155 ). In accordance with these results, strongly suggestive of a mechanism dependent on a purely electrophysiological interference on the heart, recent preliminary data from single case reports demonstrated the effectiveness of immunosuppressive therapy in reversing anti-Ro/SSA-associated electrocardiographic abnormalities in vivo , at least in adults ( 116 , 156 – 158 ).…”

Section: Autoimmunity As a Cause Of Acquired Lqtssupporting

confidence: 76%

Long QT Syndrome: An Emerging Role for Inflammation and Immunity

Lazzerini

Capecchi

Laghi‐Pasini

2015

Front. Cardiovasc. Med.

136

123

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The long QT syndrome (LQTS), classified as congenital or acquired, is a multi-factorial disorder of myocardial repolarization predisposing to life-threatening ventricular arrhythmias, particularly torsades de pointes. In the latest years, inflammation and immunity have been increasingly recognized as novel factors crucially involved in modulating ventricular repolarization. In the present paper, we critically review the available information on this topic, also analyzing putative mechanisms and potential interplays with the other etiologic factors, either acquired or inherited. Accumulating data indicate inflammatory activation as a potential cause of acquired LQTS. The putative underlying mechanisms are complex but essentially cytokine-mediated, including both direct actions on cardiomyocyte ion channels expression and function, and indirect effects resulting from an increased central nervous system sympathetic drive on the heart. Autoimmunity represents another recently arising cause of acquired LQTS. Indeed, increasing evidence demonstrates that autoantibodies may affect myocardial electric properties by directly cross-reacting with the cardiomyocyte and interfering with specific ion currents as a result of molecular mimicry mechanisms. Intriguingly, recent data suggest that inflammation and immunity may be also involved in modulating the clinical expression of congenital forms of LQTS, possibly triggering or enhancing electrical instability in patients who already are genetically predisposed to arrhythmias. In this view, targeting immuno-inflammatory pathways may in the future represent an attractive therapeutic approach in a number of LQTS patients, thus opening new exciting avenues in antiarrhythmic therapy.

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Section: Autoimmunity As a Cause Of Acquired Lqtssupporting

confidence: 76%

Long QT Syndrome: An Emerging Role for Inflammation and Immunity

Lazzerini

Capecchi

Laghi‐Pasini

2015

Front. Cardiovasc. Med.

136

123

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“…Indeed, autoantibodies critically interfere with cardiac electrical function and promote the development of life-threatening arrhythmic events by affecting the function of cardiac ion channels (15). A large number of studies have clearly demonstrated that anti-Ro/SSA autoantibody cross-reactivity with L-type Ca 2+ channels, and subsequent inhibition of I CaL in the fetal heart conduction system are crucial pathogenic mechanisms through which these autoantibodies lead to the development of ACHB (15, 23–25). Increasing evidence indicates that the hERG1 K + channel is also a specific target of anti-Ro/SSA, which constitutes a novel form of acquired long QT syndrome (LQTS) of autoimmune origin (15, 26).…”

Section: Discussionmentioning

confidence: 99%

Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center

Wang

Shi

et al. 2019

Front. Pediatr.

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Objective: To describe the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy, and to explore the therapeutic role of glucocorticoids in these diseases.Methods: This was a retrospective observational study of 2 fetuses and 14 children who presented with autoantibody-mediated arrhythmia and/or cardiomyopathy in our hospital from September 2010 to December 2018.Results: In total, 16 patients were identified, including 2 fetuses, and 14 children. One mother suffered from Sjogren's syndrome, two suffered from systemic lupus erythematosus (SLE), and the remaining 13 were asymptomatic carriers of autoantibodies. Two fetuses were diagnosed with complete congenital heart block (CHB) and had mean heart rates of 45 and 50 bpm. In the 14 surviving children, third-degree CHB was detected in 4 children, second- to third-degree CHB in 4, corrected QT interval (QTc) prolongation in 1, atrioventricular dissociation, and junctional ectopic tachycardia in 1, complete left bundle branch block (CLBBB) with dilated cardiomyopathy (DCM) in 3, and endocardial fibroelastosis (EFE) in 1. All of the 14 surviving babies received intravenous immunoglobulin and glucocorticoids. None of the children received pacemaker implantation. During the follow-up, one 3-month-old girl who had complete CHB, DCM, and Torsades de pointes almost recovered after the administration of prednisone for ~8 years. Three cases with complete CHB had no improvement after 3–5 years of follow-up. One case with EFE and three cases with CLBBB and DCM were in stable condition now. Children with QTc prolongation and junctional ectopic tachycardia returned to a regular rhythm.Conclusions: Autoantibody-mediated arrhythmias and/or cardiomyopathy are severe complications related to maternal autoantibodies, and the administration of steroid may be beneficial in reversing complete CHB.

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“…1,4,5 Regarding immunological origin, this is caused by anti-Ro, and anti-LA maternal antibodies in 85-90%, and these antibodies expressed in collagen diseases such as systemic lupus erythematosus and Sjögren's síndrome. 6 A percentage of maternal carriers of anti-Ro and anti-La no symptoms of the disease, so there is a group of women who will be pregnant without knowing the risk to the fetus; 7 fortunately only about 2% of seropositive mothers fetuses present heart condition, particularly the piping system. 5,7 To understand the pathophysiology of AV congenital blocking is necessary to understand the fundamental role of anti-Ro and anti-La antibodies, which are directed against antigens ribonucleoprotein complex present in the cells of cardiac conduction and cardiac myocytes located in the cytoplasm.…”

Section: Introductionmentioning

confidence: 99%

Fetal complete atrioventricular block: diagnostic and therapeutic approach: a case report and review of the literature

Poveda-Rojas

Vélez-Tirado

Bonilla-Cortes

et al. 2018

OGIJ

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Objective: To report a case of congenital complete atrioventricular block and perform a literature review of diagnosis and treatment. Materials and methods: The case of a pregnant 27 year old who consults a general hospital of high complexity, with a pregnancy of 33 weeks single fetus diagnosed with complete atrioventricular block and dilated cardiomyopathy secondary is reported. Prenatal management began with Betamimetic with poor response so it was necessary to end the pregnancy. The newborn requires implantation of ventricular pacemakers in the first day of life with excellent results in follow-up to 1 year. Medline, Lilacs, SciELO, by the words: literature review published in the data bases is done "fetal complete atrioventricular block", "congenital complete heart block" with limits year from 2000 to 2016, Spanish and English. Results: 21 publications were obtained; seven case reports ten literature reviews four cohort studies. The diagnosis is based on fetal echocardiography showing valvular regurgitation, myocardial/valvular hyperechogenicity-endocardial fibroelastosis, premature atrial contractions and pericardial effusion. Prenatal treatment with respect to the corticosteoides and Betamimetics, are the most commonly used medications. Treatment of severe refractory neonatal bradiarritimia may require implantation of a pacemaker as definitive management Conclusion: Congenital third-degree AV block requires early diagnosis and treatment is associated with high perinatal morbidity and mortality. More studies are needed methodological quality that allow other options to endorse promising therapeutic regimens.

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Anti-Ro/SSA antibodies and cardiac rhythm disturbances: Present and future perspectives

Cited by 16 publications

References 55 publications

Long QT Syndrome: An Emerging Role for Inflammation and Immunity

Long QT Syndrome: An Emerging Role for Inflammation and Immunity

Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center

Fetal complete atrioventricular block: diagnostic and therapeutic approach: a case report and review of the literature

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