Mediastinal IgG4-Related Disease Manifesting as Superior Vena Cava Syndrome

Matthew R DeLuzio; Negar Rassaei; Matthew D Taylor; Michael F Reed

doi:10.1016/j.athoracsur.2020.10.064

Mediastinal IgG4-Related Disease Manifesting as Superior Vena Cava Syndrome

Ann Thorac Surg. 2021 Jul;112(1):e49-e51. doi: 10.1016/j.athoracsur.2020.10.064. Epub 2021 Jan 4.

Authors

Matthew R DeLuzio¹, Negar Rassaei², Matthew D Taylor¹, Michael F Reed³

Affiliations

¹ Division of Thoracic Surgery, Department of Surgery, Penn State Health Milton S. Hershey Medical Center, Hershey, Pennsylvania.
² Department of Pathology, Penn State Health Milton S. Hershey Medical Center, Hershey, Pennsylvania.
³ Division of Thoracic Surgery, Department of Surgery, Penn State Health Milton S. Hershey Medical Center, Hershey, Pennsylvania. Electronic address: mreed2@pennstatehealth.psu.edu.

PMID: 33412141
DOI: 10.1016/j.athoracsur.2020.10.064

Abstract

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.

Publication types

Case Reports

MeSH terms

Adult
Humans
Immunoglobulin G4-Related Disease / diagnosis
Immunoglobulin G4-Related Disease / etiology*
Male
Mediastinum
Superior Vena Cava Syndrome / complications*
Superior Vena Cava Syndrome / diagnosis
Superior Vena Cava Syndrome / surgery
Tomography, X-Ray Computed