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A challenging case report of IgG4-related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions.

Authors
  • Hajsadeghi, Shokoufeh1
  • Pakbaz, Marziyeh2
  • Hassanzadeh, Morteza3
  • Sadeghipour, Alireza4
  • 1 Research Center for Prevention of Cardiovascular Disease, Institute of Endocrinology & Metabolism, Iran University of Medical Sciences, Tehran, Iran. , (Iran)
  • 2 Department of Cardiovascular Disease, Hazrat-e Rasool General Hospital, Iran University of Medical Sciences, Tehran, Iran. , (Iran)
  • 3 Department of Internal Medicine, Iran University of Medical Sciences, Tehran, Iran. , (Iran)
  • 4 Department of Pathology and Oncopathology Research Center, Iran University of Medical Sciences, Tehran, Iran. , (Iran)
Type
Published Article
Journal
Echocardiography (Mount Kisco, N.Y.)
Publication Date
Aug 25, 2020
Identifiers
DOI: 10.1111/echo.14828
PMID: 32841427
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

The IgG4-related disease is a distinct, steroid-responsive fibro-inflammatory disorder of unknown etiology. This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4-positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri-aortitis, coronary arteritis, and pericarditis. Intra-cardiac tumefactive lesions are rarely reported. Herein, we describe a challenging case of IgG4-related disease with a long-time lag between initiation of symptoms to proper diagnosis with biopsy-proven cardiac and retroperitoneal and possible pituitary gland involvement. Concerning the rarity of the cardiac lesion in our case, we conducted a literature review of similar case reports. © 2020 Wiley Periodicals LLC.

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