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Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system
  1. Peter M Izmirly1,
  2. Amit Saxena1,
  3. Sara K Sahl1,
  4. Ummara Shah2,
  5. Deborah M Friedman3,
  6. Mimi Y Kim4,
  7. Jill P Buyon1
  1. 1Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, New York, USA
  2. 2Division of Rheumatology, Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, New York, USA
  3. 3Division of Pediatric Cardiology, Department of Pediatrics, New York Medical College, Valhalla, New York, USA
  4. 4Department of Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, New York, USA
  1. Correspondence to Dr Peter M Izmirly, NYU School of Medicine, Hospital for Joint Diseases, 301 East 17th Street, Room 1611B, New York, NY 10003, USA; Peter.Izmirly{at}nyumc.org

Abstract

Objectives 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.

Methods 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.

Results 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.

Conclusions 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.

  • Outcomes research
  • Corticosteroids
  • Autoantibodies
  • Cardiovascular Disease

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