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...


Immunoglobulin G4-related disease IgG4 Aortitis Aortic aneurysm 


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

11420_2017_9565_MOESM1_ESM.pdf (1.2 mb)
ESM 1 (PDF 1224 kb)
11420_2017_9565_MOESM2_ESM.pdf (1.2 mb)
ESM 2 (PDF 1224 kb)
11420_2017_9565_MOESM3_ESM.pdf (1.2 mb)
ESM 3 (PDF 1224 kb)
11420_2017_9565_MOESM4_ESM.pdf (1.2 mb)
ESM 4 (PDF 1224 kb)
11420_2017_9565_MOESM5_ESM.pdf (1.2 mb)
ESM 5 (PDF 1224 kb)
11420_2017_9565_MOESM6_ESM.pdf (1.2 mb)
ESM 6 (PDF 1224 kb)
11420_2017_9565_MOESM7_ESM.pdf (1.2 mb)
ESM 7 (PDF 1224 kb)
11420_2017_9565_MOESM8_ESM.pdf (46 kb)
ESM 8 (PDF 46 kb)


  1. 1.
    Dalton JC, Case 22-1962. New England Journal of Medicine, 1962. 266(14): p. 719–725.CrossRefGoogle Scholar
  2. 2.
    Frulloni L, et al., Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med, 2009. 361(22): p. 2135–42.CrossRefPubMedGoogle Scholar
  3. 3.
    Hoff JA, et al., Age and gender distributions of coronary artery calcium detected by electron beam tomography in 35,246 adults. Am J Cardiol, 2001. 87(12): p. 1335–9.CrossRefPubMedGoogle Scholar
  4. 4.
    Kasashima S, et al., Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis. Am J Surg Pathol, 2008. 32(2): p. 197–204.CrossRefPubMedGoogle Scholar
  5. 5.
    Khosroshahi A, et al., Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum, 2010. 62(6): p. 1755–62.CrossRefPubMedGoogle Scholar
  6. 6.
    Khosroshahi A, et al., Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore), 2012. 91(1): p. 57–66.CrossRefGoogle Scholar
  7. 7.
    Khosroshahi A and JH Stone, Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol, 2011. 23(1): p. 67–71.CrossRefPubMedGoogle Scholar
  8. 8.
    Khosroshahi A, et al., International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol, 2015. 67(7): p. 1688–99.CrossRefPubMedGoogle Scholar
  9. 9.
    Masaki Y, et al., Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis, 2009. 68(8): p. 1310–5.CrossRefPubMedGoogle Scholar
  10. 10.
    Monach PA, IgG4-related disease: 2013 update. Curr Treat Options Cardiovasc Med, 2013. 15(2): p. 214–23.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Najari BB, et al., Clinical, radiographic, and pathologic description of IgG4-related perivasal fibrosis: a previously undescribed etiology of chronic orchialgia. Urology, 2014. 84(4): p. 748–50.CrossRefPubMedGoogle Scholar
  12. 12.
    Nishimori I, A Tamakoshi, M Otsuki, Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. J Gastroenterol, 2007. 42 Suppl 18: p. 6–8.CrossRefPubMedGoogle Scholar
  13. 13.
    Paravastu SC, et al., A systematic review of open versus endovascular repair of inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg, 2009. 38(3): p. 291–7.CrossRefPubMedGoogle Scholar
  14. 14.
    Perugino CA, et al., Large vessel involvement by IgG4-related disease. Medicine (Baltimore), 2016. 95(28): p. e3344.Google Scholar
  15. 15.
    Raina A, et al., Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol, 2009. 104(9): p. 2295–306.CrossRefPubMedGoogle Scholar
  16. 16.
    Rojo-Leyva F, et al., Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases. Arthritis Rheum, 2000. 43(4): p. 901–7.CrossRefPubMedGoogle Scholar
  17. 17.
    Stone JH, et al., IgG4-related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res (Hoboken), 2010. 62(3): p. 316–22.CrossRefGoogle Scholar
  18. 18.
    Stone JH, Y Zen, V Deshpande, IgG4-related disease. N Engl J Med, 2012. 366(6): p. 539–51.CrossRefPubMedGoogle Scholar
  19. 19.
    Stone WM, et al., Comparison of open and endovascular repair of inflammatory aortic aneurysms. J Vasc Surg, 2012. 56(4): p. 951–5; discussion 955-6.CrossRefPubMedGoogle Scholar
  20. 20.
    Waheed S, et al., Collective impact of conventional cardiovascular risk factors and coronary calcium score on clinical outcomes with or without statin therapy: the St Francis Heart Study. Atherosclerosis, 2016. 255: p. 193–199.CrossRefPubMedGoogle Scholar
  21. 21.
    Zen Y Y Nakanuma, IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol, 2010. 34(12): p. 1812–9. CrossRefPubMedGoogle Scholar

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

Personalised recommendations