IgG4-related aortitis mimicking acute aortic and coronary syndromes, multimodality imaging–pathology correlation: a case report - Report - MDSpire

IgG4-related aortitis mimicking acute aortic and coronary syndromes, multimodality imaging–pathology correlation: a case report

  • By

  • Laura Victoria Torres-Araujo

  • Valente Fernandez-Badillo

  • Antonio Jordan-Rios

  • Rodrigo Gopar-Nieto

  • Silvia Jimenez-Becerra

  • Jesus A. Cotes-Millan

  • Daniel Sierra Lara-Martinez

  • Benjamin I. Hernandez-Mejia

  • Moises Jimenez-Santos

  • Sergio A. Criales-Vera

  • June 4, 2026

  • 0 min

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Clinical Report: IgG4-Related Aortitis Mimicking Acute Aortic Syndromes

Overview

This case report discusses a 64-year-old man with IgG4-related aortitis that presented as acute aortic and coronary syndromes. The diagnostic challenge posed by this condition is highlighted, emphasizing the need for awareness among clinicians.

Background

IgG4-related aortitis is a rare inflammatory condition that can closely resemble acute aortic syndromes, complicating diagnosis in patients with chest pain. Its under-recognition in acute cardiovascular settings can lead to misdiagnosis and inappropriate management. Understanding this condition is crucial for timely and accurate diagnosis to improve patient outcomes.

Data Highlights

No numerical data provided in the article.

Key Findings

  • IgG4-related aortitis can mimic both acute aortic syndromes and acute coronary syndromes.
  • Imaging features such as concentric wall thickening can lead to diagnostic misclassification.
  • Histopathological examination remains the gold standard for diagnosis when imaging is inconclusive.
  • Multimodal imaging may not reliably distinguish between inflammatory and structural aortic pathologies in acute settings.
  • Current diagnostic algorithms do not account for inflammatory aortitides like IgG4-RD.

Clinical Implications

Clinicians should maintain a high index of suspicion for IgG4-related aortitis in patients presenting with acute chest pain, especially when imaging findings are discordant with clinical presentation. Awareness of this condition can aid in preventing misdiagnosis and inappropriate surgical interventions.

Conclusion

This case underscores the critical need for heightened awareness of IgG4-related aortitis in acute cardiovascular care to improve diagnostic accuracy and patient management.

Related Resources & Content

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  2. Clinical Rheumatology, 2007 -- The Importance of PET/CT Imaging in Diagnosing Cogan’s Syndrome
  3. Clinical Rheumatology, 2025 -- A Case Review of Takayasu Arteritis Associated with SAPHO Syndrome
  4. Hypertrophic Pachymeningitis Associated with IgG4-Related Disease Featuring Tumor-like Lesions in the Brain
  5. Multimodality Imaging Features of Immunoglobulin G4–related Vessel Involvement - PMC
  6. Inebilizumab for Treatment of IgG4-Related Disease | New England Journal of Medicine
  7. 2025 ACC/AHA/ACEP/NAEMSP/SCAI Guideline for the Management of Patients With Acute Coronary Syndromes
  8. Multimodality Imaging Features of Immunoglobulin G4–related Vessel Involvement - PMC
  9. Inebilizumab for Treatment of IgG4-Related Disease | New England Journal of Medicine
  10. 2025 ACC/AHA/ACEP/NAEMSP/SCAI Guideline for the Management of Patients With Acute Coronary Syndromes: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines | JACC

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