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Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis?

Alessandra Palmisano1, Maria L Urban1, Domenico Corradi2

  • 1Nephrology Unit, University Hospital of Parma.

Rheumatology (Oxford, England)
|June 25, 2015
PubMed

Insights

Chronic periaortitis (CP) often affects the thoracic aorta and epiaortic arteries in about one-third of patients. This finding supports CP as a systemic inflammatory disease affecting large arteries.

Area of Science:

  • Vascular Medicine
  • Rheumatology
  • Cardiology

Background:

  • Chronic periaortitis (CP) is a rare fibro-inflammatory condition typically involving the abdominal aorta and iliac arteries.
  • Anecdotal evidence suggests CP may extend to other vascular areas, including the thoracic aorta.

Purpose of the Study:

  • To investigate the extent of thoracic aorta and epiaortic artery involvement in patients diagnosed with chronic periaortitis.
  • To compare clinical characteristics between CP patients with and without thoracic disease.

Main Methods:

  • Retrospective analysis of 153 CP patients (1999-2012) who underwent thoracic aorta and epiaortic artery imaging (CT, MRI, PET-CT).
  • Exploration of thoracic vessel involvement patterns and comparison of clinical features.
  • Review of available thoracic vascular/perivascular tissue biopsies.

Main Results:

  • Of 77 eligible CP patients, 28 (36%) exhibited thoracic involvement.
  • Thoracic involvement included thoracic periaortitis (54%), periaortitis with thoracic aortic aneurysm (21%), and thoracic aortic aneurysm alone (25%).
  • Patients with thoracic disease were more often female, older, and presented with pain and constitutional symptoms.

Conclusions:

  • Chronic periaortitis frequently extends to the thoracic aorta and epiaortic arteries in approximately one-third of cases.
  • The observed patterns in thoracic biopsies mirror those found in abdominal CP.
  • These findings support the classification of CP as a systemic inflammatory disease impacting large arteries.
Abstract

Keywords:
aneurysmsaortagiant cell arteritisperiaortitisretroperitoneal fibrosisvasculitis

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