Clinically isolated aortitis: imaging features and clinical outcomes: comparison with giant cell arteritis and giant cel

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ORIGINAL PAPER

Clinically isolated aortitis: imaging features and clinical outcomes: comparison with giant cell arteritis and giant cell aortitis Ayaz Aghayev1   · Camden P. Bay2 · Sara Tedeschi3 · Paul A. Monach3 · Umberto Campia4 · Marie Gerhard‑Herman4 · Michael L. Steigner1 · Richard N. Mitchell5 · William P. Docken3 · Marcelo DiCarli6 Received: 1 October 2020 / Accepted: 23 October 2020 © Springer Nature B.V. 2020

Abstract (1) describe imaging features of CIA, (2) compare dilation rate and wall thickening of aortic aneurysms in patients with CIA versus those with giant cell arteritis/aortitis (GCA), (3) present clinical outcomes of CIA patients. Retrospective search of electronic records from 2004 to 2018 yielded 71 patients, 52 of whom were female, with a mean age of 67.5 ± 9.0 years old, with a new clinical diagnosis of cranial or extracranial GCA (GCA group), and giant cell aortitis revealed by the aortic biopsy (CIA group). Comparisons between groups were conducted using the Wilcoxon rank-sum and Fisher’s exact tests. Survival from the date of initial diagnosis to the end of data collection was compared between the two groups through a log-rank test. CIA patients (n = 23; 32%) presented with cardiovascular symptoms, and none had systemic inflammatory symptoms. Inflammatory markers were significantly higher among GCA patients than among CIA patients (p < 0.0001). The CIA group demonstrated thoracic aortic aneurysms without wall thickening. None of the GCA patients (n = 48; 68%) had aneurysmal dilation in the aorta at the time of diagnosis. None of the four CIA patients had FDG uptake in the aorta, while nine out of 13 GCA patients had FDG uptake in the vessels. There was no statistically significant difference in the survival between the two groups (p = 0.12). CIA patients presented with cardiovascular symptoms and was characterized by aneurysm of the aorta without the involvement of the infrarenal aortic segment. The role of FDG-PET/CT in CIA is less certain, though none of the patients in this cohort had FDG uptake in the vessels. Keywords  Clinically isolated aortitis · Giant cell arteritis · Computed tomography angiography · Magnetic resonance angiography · FDG-PET/CT

Introduction * Ayaz Aghayev [email protected] 1



Cardiovascular Imaging Program, Department of Radiology, Brigham and Women’s Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA

2



Department of Radiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

3

Division of Rheumatology, Immunology and Allergy, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

4

Department of Medicine, Cardiovascular Division, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

5

Department of Pathology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, USA

6

Cardiovascular Imaging Program, Departments of Medicine and Radiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA







Aortitis is inflammation of the