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Original research article
IgG4-aortitis among thoracic aortic aneurysms
  1. Carlos Nicolás Pérez-García1,
  2. Carmen Olmos1,
  3. David Vivas1,
  4. Carlos Ferrera1,
  5. Daniel García-Arribas1,
  6. Daniel Enríquez-Vázquez1,
  7. Manuel Carnero-Alcázar1,
  8. Luis Maroto1,
  9. Aida Ortega Candil2,
  10. Melchor Saiz-Pardo Sanz3,
  11. Ana Bustos4,
  12. Luis Ortega3,
  13. Isidre Vilacosta1
  1. 1 Cardiovascular Institute, Hospital Clínico Universitario San Carlos, Madrid, Spain
  2. 2 Nuclear Medicine, Hospital Clínico Universitario San Carlos, Madrid, Spain
  3. 3 Pathology Department, Hospital Clínico Universitario San Carlos, Madrid, Spain
  4. 4 Radiology Department, Hospital Clínico Universitario San Carlos, Madrid, Spain
  1. Correspondence to Dr Carlos Nicolás Pérez-García, Cardiovascular Institute, Hospital Clinico Universitario San Carlos, 28040 Madrid, Spain;{at}


Objective The incidence of aortitis in patients with thoracic aortic diseases is not well established. The aim of this study was to analyse the frequency and clinical course of patients with aortitis in a surgical series.

Methods 320 consecutive patients with ascending aorta/aortic arch aneurysm or acute aortic syndrome who underwent surgery from 2012 to 2017 in a single tertiary referral hospital were retrospectively reviewed. Epidemiological data, clinical course and variables related to diagnosis, treatment and follow-up were collected from patients with histologically proven aortitis.

Results From 320 examined aortic samples, 279 (87.2%) thoracic aneurysms and 41 acute aortic syndromes (12.8%), 9 (2.8%) were aortitis: 3 cases of Takayasu’s arteritis, 3 of IgG4-related aortitis, 2 of giant cell, and 1 classified as idiopathic. Median age at surgery was 53.4 (51–69.2) years and six cases were female. Seven patients presented with non-specific symptoms and the diagnosis was made at pathology. Surgery was elective in eight patients and emergent in one case of IgG4-related aortitis. 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) was performed for disease extension study and as a monitoring technique during the follow-up of five patients, with just one case performed presurgically. All the patients with IgG4-related disease showed extrathoracic aortic involvement. There were no deaths, neither in-hospital nor during the 1.7 years of median follow-up.

Conclusions In surgically treated thoracic aorta pathology, the frequency of aortitis is low; IgG4-related disease is among the most common aetiologies with a frequency similar to other types of aortitis, such as Takayasu’s and giant cell arteritis, and clinical manifestations are non-specific making presurgical diagnosis difficult. 18F-FDG PET/CT allows a better assessment of disease extension and therapeutic response. Surgery can be successfully performed and corticosteroid therapy ensures a good mid-term follow-up.

  • aortic surgery
  • aortic aneurysm
  • aortitis

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  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Ethics approval The study was approved by the local ethical committees and informed consent was obtained from each patient.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.

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