IgG4-Related Disease Causing Rapid Evolution of a Severe Aortic Valvular Stenosis.

Bruls, Samuel; Courtois, Audrey; Delvenne, Philippe; Hustinx, Roland; Moutschen, Michel; De Leval, Laurence; Defraigne, Jean-Olivier; Sakalihasan, Natzi · Ann Thorac Surg · 2017

case_report · Level V

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Abstract

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recognized emerging entity characterized by chronic fibroinflammation that can affect every organ but rarely affects the cardiovascular system. We report a rare case of IgG4-RSD involving an aortic valve that resulted in rapid progression of an aortic valvular stenosis and was successfully treated by aortic valve replacement and corticosteroids.

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