Article Text

Download PDFPDF
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}

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


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 …

View Full Text


  • Handling editor Josef S Smolen

  • Contributors All authors drafted and revised the manuscript. All authors read and approved the final manuscript.

  • Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Next of kin consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.