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IgG4-related disease of the mitral valve demonstrated by immunohistochemistry
  1. Benedict K Tiong1,2,
  2. Gregory A Fishbein3,
  3. Ernest Brahn1
  1. 1 Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, USA
  2. 2 United States Department of Veterans Affairs, Los Angeles, CA, USA
  3. 3 Department of Pathology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, USA
  1. Correspondence to Dr Benedict K Tiong; btiong{at}mednet.ucla.edu

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Introduction

IgG4-related disease (IgG4-RD) is known to involve the cardiovascular system as aortitis or periaortitis1; however, the number of reported valvulopathies are exceptionally rare. This case is the first with immunohistochemistry proven mitral valve involvement.

Case report

A 72-year-old Korean woman with paroxysmal atrial fibrillation and end-stage renal disease due to long-standing diabetes and hypertension presented to the emergency department with near-syncope and dyspnoea with exertion. Further evaluation revealed three-vessel coronary artery disease and severe mitral valve stenosis due to suspected rheumatic mitral valve disease with a mitral valve area of 0.62 cm2, peak gradient of 15.5 mm Hg and mean velocity of 136 cm/s. The patient underwent three-vessel coronary artery bypass graft surgery and mitral valve replacement with a 27 mm pericardial bioprosthesis. Both the anterior and posterior chordae tendineae were …

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