Mediastinal IgG4-Related Disease Manifesting as Superior Vena Cava Syndrome

Immunoglobulin G4 (IgG4)–related disease was first identified as a systemic condition in 2003 when extrapancreatic manifestations were identified in patients with autoimmune pancreatitis. Its peak incidence occurs in the fifth or sixth decades of life. Isolated extraaortic mediastinal involvement is extremely rare. This report describes a case of isolated extraaortic mediastinal IgG4-related disease encasing the superior vena cava (SVC) and manifesting as SVC syndrome in a 25-year-old man with no personal or family history of autoimmune disease. Resection with SVC reconstruction was performed.

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Work Title Mediastinal IgG4-Related Disease Manifesting as Superior Vena Cava Syndrome
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Open Access
Creators
  1. Matthew R. DeLuzio
  2. Negar Rassaei
  3. Matthew D. Taylor
  4. Michael F. Reed
License CC BY-NC-ND 4.0 (Attribution-NonCommercial-NoDerivatives)
Work Type Article
Publisher
  1. The Annals of Thoracic Surgery
Publication Date June 18, 2021
Publisher Identifier (DOI)
  1. https://doi.org/10.1016/j.athoracsur.2020.10.064
Deposited February 19, 2024

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Version 1
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  • Created
  • Added Mediastinal_IgG-4_Pre-proof.pdf
  • Added Creator Matthew R. DeLuzio
  • Added Creator Negar Rassaei
  • Added Creator Matthew D. Taylor
  • Added Creator Michael F. Reed
  • Published
  • Updated Publisher, Publication Date Show Changes
    Publisher
    • Annals of Thoracic Surgery
    • The Annals of Thoracic Surgery
    Publication Date
    • 2021-07-01
    • 2021-06-18
  • Updated