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HSS Journal ®

, Volume 13, Issue 3, pp 307–312 | Cite as

Immunoglobulin G4-related Aortitis

A Clinical Pathology Conference Held by the Division of Rheumatology at the Hospital for Special Surgery
  • Ozan Unlu
  • Elizabeth Schulman
  • Steven D. Wolff
  • Navneet Narula
  • John H. Stone
  • Anne Bass
  • Doruk Erkan
Clinical Pathology Conference

Clinical Presentation

A 62-year-old woman presented with progressive exertional substernal chest pain for 1 year. A cardiac computerized tomography angiography (CTA) was performed, demonstrating focal high-grade stenosis and possible occlusion of the middle portion of the left anterior descending (LAD) artery, as well as a stenosis with multifocal calcification/plaque in the proximal and middle right coronary artery (RCA). An ascending aortic aneurysm with intramural hematoma measuring 4.1 × 3.7 cm was also detected. The patient had a coronary artery calcium score (CAC) of 429. The CAC score predicts cardiovascular outcomes independent of traditional cardiovascular risk profile [ 3, 20] and the patient’s calcium score corresponded to 97th percentile showing a high risk of coronary atherosclerotic plaque presence [ 3]. She subsequently underwent a cardiac catheterization, which confirmed three-vessel coronary disease (LAD, RCA, and diagonal 2). One week later, she underwent a...

Keywords

Immunoglobulin G4-related disease IgG4 Aortitis Aortic aneurysm 

Notes

Compliance with Ethical Standards

Conflict of Interest

Ozan Unlu, MD; Elizabeth Schulman, MD; Steven D. Wolff, MD, PhD; Navneet Narula, MD; John H. Stone, MD, PhD; Anne Bass, MD; and Doruk Erkan, MD, MPH have declared that they have no conflict of interest.

Human/Animal Rights

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008.

Informed Consent

Informed consent was waived from all patients for being included in the study.

Required Author Forms

Disclosure forms provided by the authors are available with the online version of this article.

Supplementary material

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Copyright information

© Hospital for Special Surgery 2017

Authors and Affiliations

  1. 1.Division of RheumatologyHospital for Special SurgeryNew YorkUSA
  2. 2.Division of RheumatologyWeill Cornell MedicineNew YorkUSA
  3. 3.Carnegie Hill RadiologyNew YorkUSA
  4. 4.Clinical Pathology and Laboratory MedicineNew York Presbyterian HospitalNew YorkUSA
  5. 5.Clinical Pathology and Laboratory MedicineWeill Cornell MedicineNew YorkUSA
  6. 6.Division of Rheumatology, Allergy & Immunology, Massachusetts General HospitalHarvard Medical SchoolBostonUSA

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