title: Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system.
abstract: Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit.
  
  
  
  In this retrospective study of cases enrolled in the Research Registry for NL, inclusion was restricted to anti-SSA/Ro-exposed cases presenting with isolated advanced heart block in utero who either received fluorinated steroids within 1 week of detection (N=71) or no treatment (N=85). Outcomes evaluated were: development of endocardial fibroelastosis, dilated cardiomyopathy and/or hydrops fetalis; mortality and pacemaker implantation.
  
  
  
  In Cox proportional hazards regression analyses, fluorinated steroids did not significantly prevent development of disease beyond the AV node (adjusted HR=0.90; 95% CI 0.43 to 1.85; p=0.77), reduce mortality (HR=1.63; 95% CI 0.43 to 6.14; p=0.47) or forestall/prevent pacemaker implantation (HR=0.87; 95% CI 0.57 to 1.33; p=0.53). No risk factors for development of disease beyond the AV node were identified.
  
  
  
  These data do not provide evidence to support the use of fluorinated steroids to prevent disease progression or death in cases presenting with isolated heart block.
authors.affiliation: Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, New York, USA.
authors.forename: Peter M
authors.initials: PM
authors.lastname: Izmirly
authors.affiliation: Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, New York, USA.
authors.forename: Amit
authors.initials: A
authors.lastname: Saxena
authors.affiliation: Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, New York, USA.
authors.forename: Sara K
authors.initials: SK
authors.lastname: Sahl
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, New York, USA.
authors.forename: Ummara
authors.initials: U
authors.lastname: Shah
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, New York Medical College, Valhalla, New York, USA.
authors.forename: Deborah M
authors.initials: DM
authors.lastname: Friedman
authors.affiliation: Department of Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, New York, USA.
authors.forename: Mimi Y
authors.initials: MY
authors.lastname: Kim
authors.affiliation: Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, New York, USA.
authors.forename: Jill P
authors.initials: JP
authors.lastname: Buyon
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood*; D018450:Disease Progression; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000401:mortality; D006327:Heart Block / Q000151:congenital / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D006801:Humans; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000151:congenital; D008297:Male; D010138:Pacemaker, Artificial; D011295:Prenatal Care / Q000379:methods; D012042:Registries; D012189:Retrospective Studies; D013259:Steroids, Fluorinated / Q000627:therapeutic use*; D016216:Ultrasonography, Prenatal; D014481:United States / Q000453:epidemiology
chemical_list: D000974:Antibodies, Antinuclear; C035356:SS-A antibodies; D013259:Steroids, Fluorinated
keywords: Autoantibodies; Cardiovascular Disease; Corticosteroids; Outcomes research
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