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Libman–Sacks endocarditis as the first manifestation of systemic lupus erythematosus in an adolescent, with a review of the literature

Published online by Cambridge University Press:  18 July 2012

Jayendra Sharma*
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Jamaica Hospital Medical Center, Jamaica
Zoran Lasic
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Jamaica Hospital Medical Center, Jamaica
Abraham Bornstein
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Cohen Children's Hospital of New York, Hofstra North Shore LIJ School of Medicine, New York, United States of America
Rubin Cooper
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Cohen Children's Hospital of New York, Hofstra North Shore LIJ School of Medicine, New York, United States of America
Jonathan Chen
Affiliation:
Division of Pediatric Cardiology and Cardiothoracic Surgery, Department of Pediatrics, The New York Presbyterian Hospital – Weill Medical College, New York, New York, United States of America
*
Correspondence to: Dr J. Sharma, MD, FACC, Division of Pediatric Cardiology, Department of Pediatrics, Jamaica Hospital Medical Center, 8900 Van Wyck Expressway, Jamaica, New York 11418, United States of America. Tel: +718 206 7138; Fax: +718 206 7144; E-mail: jsharma@jhmc.org

Abstract

Libman–Sacks endocarditis is rare in children and adolescents, more so as a first manifestation of systemic lupus erythematosus. Currently, sterile verrucous lesions of Libman–Sacks endocarditis are recognised as a cardiac manifestation of both systemic lupus erythematosus and antiphospholipid syndrome. They are clinically silent in a majority of the cases. The presence of antiphospholipid antibodies in systemic lupus erythematosus is associated with three times higher prevalence of mitral valve nodules and significant mitral regurgitation. We present the case of isolated mitral regurgitation with abnormal looking mitral valve, detected in early childhood, which deteriorated to a severe degree in the next decade and was diagnosed as Libman–Sacks endocarditis after surgical repair from histopathology. The full-blown clinical spectrum of systemic lupus erythematosus with antiphospholipid antibodies was observed several weeks after cardiac surgery. We discuss the atypical course of Libman–Sacks endocarditis with follow-up for 10 years, along with a review of the literature.

Type
Review Article
Copyright
Copyright © Cambridge University Press 2012

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